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POROQUERATOSIS, ACTUALIZACION
POROKERATOSIS, UPDATE 
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***** DERMAGIC-EXPRESS No.29 ******** 
****** 13 ENERO DE 1.999 ********* 
***** 13 JANUARY 1.999 ************ 
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EDITORIAL ESPANOL
=================
Hola amigos DERMAGICOS, el tema de hoy, LA POROKERATOSIS. La Dra. Ilse de Santiago, solicita información sobre tratamientos. Realmente como lo expresa en su correo, hay poco sobre como tratar esta patología, pero me pareció tan interesante el tema cuando estaba buscando información, que quiza la dificultad en el tratamiento de LA POROQUERATOSIS este en sus posibles causas, por ello hice una revision completa.

Les traigo 75 referencias interesantes que nos describen bien la enfermedad, sus variantes(56) y sus posibles tratamientos (19) 

Saludos,,,

Dr. Jose Lapenta.

EDITORIAL ENGLISH
=================
Hello DERMAGIC friends, today's topic, THE POROKERATOSIS. I found so interesting the topic when I was looking for information, that maybe the difficulty in the treatment of THE POROQUERATOSIS is in their possible causes, for I made it a complete revision. 

I bring 75 interesting references that describe us well the illness, their types (56) and their possible treatments (19) 

Greetings,,,
Dr. Jose Lapenta,
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DERMAGIC/EXPRESS(29)
======================================================================
POROQUERATOSIS, LA ENFERMEDAD// POROKERATOSIS, THE DISEASE ======================================================================
1.) A report of 31 cases of porokeratosis at the National Skin Centre.
2.) Familial craniosynostosis, anal anomalies, and porokeratosis: CAP syndrome.
3.) Disseminated superficial porokeratosis in a patient with chronic liver disease.
4.) A case of disseminated superficial actinic porokeratosis subsequent to renal transplantation.
5.) High incidence of porokeratosis in renal transplant recipients.
6.) Disseminated superficial porokeratosis after autologous bone marrow transplantation.
7.) Cancer proneness of linear porokeratosis may be explained by allelic loss.
8.) Porokeratotic palmoplantar keratoderma discreta--a new entity or a variant of porokeratosis plantaris discreta?
9.) Disseminated superficial porokeratosis developing after electron-beam total skin irradiation for mycosis fungoides.
10.) Disseminated superficial porokeratosis with amyloid deposition.
11.) Homozygote erythropoietic protoporphyria associated with porokeratosis]
12.) [Mibelli porokeratosis in 2 brothers]
13.) Linear porokeratosis presenting as erosions in the newborn period.
14.) Genital porokeratosis of Mibelli.
15.) Disseminated epidermolytic acanthoma with disseminated superficial porokeratosis and verruca vulgaris in an immunosuppressed patient.
16.) Rapid development of disseminated superficial porokeratosis after transplant induction therapy.
17.) Porokeratosis and Crohn's disease.
18.) Porokeratosis and immunosuppression.
19.) Porokeratosis of Mibelli associated with active chronic hepatitis and vitiligo.
20.) Disseminated superficial actinic porokeratosis: experimental induction and exacerbation of skin lesions.
21.) Disseminated superficial porokeratosis in a patient with AIDS.
22.) Elevated chromosome aberration frequency after X-ray exposure of cultured fibroblasts derived from patients with porokeratosis.
23.) Overexpression of p53 tumor suppressor protein in porokeratosis [see comments]
24.) [Squamous cell carcinoma and disseminated superficial actinic porokeratosis]
25.) The coexistence of linear and giant porokeratosis associated with Bowen's disease.
26.) Porokeratosis in immunosuppressed and nonimmunosuppressed patients.
27.) Eruptive pruritic papular porokeratosis.
28.) Porokeratosis palmaris et plantaris disseminata. Report of a case with abnormal DNA ploidy in lesional epidermis.
29.) Porokeratosis of Mibelli following heart transplant [see comments]
30.) Linear and punctate porokeratosis associated with end-stage liver disease.
31.) Morphogenesis of the cornoid lamella: histochemical, immunohistochemical, and ultrastructural study of porokeratosis.
32.) Porokeratosis arising in a burn scar.
33.) Disseminated superficial actinic porokeratosis. A histological review of 61 cases 
34.) Superficial actinic porokeratosis and cystic fibrosis.
35.) [Linear porokeratosis of Mibelli in monozygotic twin girls]
36.) [Mibelli's porokeratosis gigantea]
37.) Disseminated porokeratosis in an infant with craniosynostosis.
38.) Disseminated porokeratosis accompanying multicentric Bowen's disease. 
39.) Unusual presentation of porokeratosis palmaris, plantaris et disseminata.
40.) Clonal chromosome abnormalities with preferential involvement of chromosome 3 in patients with porokeratosis of Mibelli.
41.) Zosteriform porokeratosis: a report of two cases.
42.) Porokeratosis punctata palmaris et plantaris. A new entity?
43.) Porokeratosis as a premalignant condition of the skin. Cytologic demonstration of abnormal DNA ploidy in cells of the epidermis.
44.) Porokeratosis of mibelli in transplant recipients.
45.) Disseminated superficial porokeratosis and immunosuppression.
46.) Linear porokeratosis in two families with disseminated superficial actinic porokeratosis.
47.) Malignant disseminated porokeratosis.
48.) Disseminated bilateral hyperkeratotic variant of porokeratosis Mibelli.
49.) Porokeratosis plantaris, palmaris, et disseminata.
50.) Porokeratosis of Mibelli: benzylhydrochlorothiazide-induced new lesions accompanied by eosinophilic spongiosis.
51.) Congenital unilateral punctate porokeratosis.
52.) Punctate porokeratotic keratoderma--its occurrence with internal neoplasia.
53.) Generalized eruptive porokeratosis of Mibelli with associated psoriasis.
54.) Cultured skin fibroblasts derived from three patients with disseminated superficial actinic porokeratosis (DSAP) are hypersensitive to the lethal effects of X-radiation but not to those of ultraviolet (UV) light.
55.) Disseminated superficial porokeratosis in patients with pemphigus vulgaris 
56.) Disseminated superficial porokeratosis developing after electron-beam total skin irradiation for mycosis fungoides.
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POROKERATOSIS, EL TRATAMIENTO /// POROKERATOSIS THE TREATMENT
======================================================================
1.) Chemotherapy for disseminated actinic keratoses with 5-fluorouracil and isotretinoin.
2.) Porokeratosis of Mibelli: rapid recurrence of a large lesion after carbon dioxide laser treatment.
3.) Generalized linear porokeratosis treated with etretinate [letter]
4.) Porokeratosis plantaris, palmaris, et disseminata. Tigason Therapy
5.) Dexamethasone pulse treatment in disseminated porokeratosis of Mibelli [letter]
6.) Treatment of porokeratosis of Mibelli with CO2 laser vaporization versus surgical excision with split-thickness skin graft. A comparison.
7.) Disseminated superficial actinic porokeratosis responding to calcipotriol [letter]
8.) Reticulate porokeratosis--successful treatment with CO2-laser vaporization.
9.) Spiny keratoderma of the palms and soles.
10.) Porokeratosis of Mibelli with underlying hemangioma treated by the flashlamp-pumped pulsed dye laser.
11.) Linear porokeratosis: successful treatment with diamond fraise dermabrasion.
12.) Digitate keratoses--a complication of etretinate used in the treatment of disseminated superficial actinic porokeratosis.
13.) Exacerbation of porokeratosis during etretinate therapy.
14.) Etretinate in the treatment of disseminated porokeratosis of Mibelli.
15.) Carbon dioxide laser treatment of porokeratosis of Mibelli.
16.) Porokeratosis (Mibelli): treatment with topical 5-fluorouracil.
17.) [Ultrastructural study of Mibelli's porokeratosis. Pathogenic and therapeutic considerations in three cases]
18.) Cryosurgery of porokeratosis plantaris discreta.
19.) Disseminated superficial porokeratosis: Complete remission subsequent to discontinuation of immunosuppression
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POROQUERATOSIS, LA ENFERMEDAD// POROKERATOSIS, THE DISEASE ======================================================================
======================================================================
1.) A report of 31 cases of porokeratosis at the National Skin Centre.
======================================================================
Leow YH; Soon YH; Tham SN
National Skin Centre, Singapore.
Ann Acad Med Singapore (SINGAPORE) Nov 1996 25 (6) p837-41 ISSN: 0304-4602
Language: ENGLISH
Document Type: JOURNAL ARTICLE 
Journal Announcement: 9707
Subfile: INDEX MEDICUS
Porokeratosis is a well-recognised disorder of keratinization with distinctive 
clinical features and histological hallmark of cornoid lamella. There are at least 4 
different clinical variants, with malignant transformation reported in almost all 
types of porokeratosis. This is a retrospective study on all cases of porokeratosis 
seen at the National Skin Centre, Singapore from 1990 to 1993. There was a total of 
31 patients diagnosed to have porokeratosis during the study period. They can be 
classified into 4 main clinical variants: (1) disseminated superficial actinic 
porokeratosis (41.9%), (2) classical porokeratosis of Mibelli (35.5%), (3) 
porokeratosis palmaris, plantaris et disseminatum (9.7%), and (4) linear 
porokeratosis (12.9%). Our typical patient is in his/her early forties, who noticed 
asymptomatic porokeratotis lesion on sun-exposed skin. Various treatment modalities 
were used, with no one method being more superior to another. None of our patients 
had malignant transformation of pre-existing skin lesions during the short follow-up 
period from less than one year to three years. Patients should be advised to avoid 
excessive sunlight, to use sunscreen and go for periodic examination by a 
dermatologist with a view to close skin malignancy surveillance.


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2.) Familial craniosynostosis, anal anomalies, and porokeratosis: CAP syndrome.
========================================================================

J Med Genet 1998 Sep;35(9):763-6 (ISSN: 0022-2593)

Flanagan N; Boyadjiev SA; Harper J; Kyne L; Earley M; Watson R; Jabs EW; Geraghty MT [Find
other articles with these Authors]

Department of Dermatology, St James Hospital, Dublin, Ireland.

We report on the occurrence of coronal craniosynostosis, anal anomalies, and porokeratosis in
two male sibs. A third male sib was phenotypically normal as were the parents. The occurrence of
these three clinical features has, to our knowledge, not been reported before. Cutaneous or anal
anomalies or both have been reported in a number of syndromes associated with craniosynostosis,
including Crouzon, Pfeiffer, Apert, and Beare-Stevenson syndromes. These syndromes are
associated with mutations in the fibroblast growth factor receptor genes FGFR1, FGFR2, and
FGFR3. They are inherited in an autosomal dominant fashion. In contrast, the cases we report do
not carry any of the common FGFR mutations and the pedigree suggests autosomal or X linked
recessive inheritance. 

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3.) Disseminated superficial porokeratosis in a patient with chronic liver disease.
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J Dermatol 1997 Jul;24(7):485-7 (ISSN: 0385-2407)

Park BS; Moon SE; Kim JA [Find other articles with these Authors]

Department of Dermatology, Seoul National University College of Medicine, Korea.

A 55-year-old male suffering from liver cirrhosis presented with diffuse annular hyperkeratotic
papules of abrupt onset on the trunk and extremities. Histopathologic examination revealed cornoid
lamella and eosinophilic spongiosis. He did not receive any medications other than cephalosporin for
spontaneous bacterial peritonitis. A review of the literature revealed that three cases developed
porokeratosis when their liver function declined and that, in one case, the porokeratosis
disappeared spontaneously with liver transplantation. Although the precise mechanism is unclear,
there is evidence demonstrating immunoincompetence in cirrhosis. Even though we did not perform
immunologic studies or exclude the possibility of drug-induced porokeratosis in our case, it is
conceivable that porokeratosis can be triggered by immunosuppression due to liver cirrhosis per
se. 

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4.) A case of disseminated superficial actinic porokeratosis subsequent to renal transplantation.
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J Dermatol 1997 Feb;24(2):110-2 (ISSN: 0385-2407)

Matsushita S; Kanekura T; Kanzaki T 
Department of Dermatology, Kagoshima University Faculty of Medicine, Japan.

Disseminated superficial actinic porokeratosis is characterized by the development of numerous
annular keratotic lesions on sun-exposed areas, accompanied by pathological evidence of cornoid
lamellae. We examined a case of disseminated superficial actinic porokeratosis in a 40-year-old
male who had undergone renal transplantation and was being treated with immunosuppressants.
Five years after surgery, he began to develop numerous eruptions. Some of these eruptions enlarged
and developed over a second period of five years until he finally required hospitalization. 

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5.) High incidence of porokeratosis in renal transplant recipients.
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Br J Dermatol 1997 Feb;136(2):176-9 (ISSN: 0007-0963)

Herranz P; Pizarro A; De Lucas R; Robayna MG; Rubio FA; Sanz A; Contreras F; Casado M
[Find other articles with these Authors]

Department of Dermatology, La Paz University Hospital, Autonoma University, Madrid, Spain.

Immunosuppression is a well-documented precipitant of porokeratosis (PK). However, PK is not
considered among the most common cutaneous disorders in immunosuppressed patients. We
studied prospectively a series of 103 renal transplant patients and found 11 cases (10.68%) of PK.
Our series represents the highest incidence of PK in transplant patients reported so far. Our findings
suggest that PK in transplant recipients may be more frequent than previously thought. 

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6.) Disseminated superficial porokeratosis after autologous bone marrow transplantation.
========================================================================
Bone Marrow Transplant 1997 Jan;19(1):77-9 (ISSN: 0268-3369)

Rio B; Magana C; Le Tourneau A; Bachmeyer C; Levy V; Hamont N; Diebold J; Zittoun R [Find
other articles with these Authors]

Service d'Hematologie, Hotel-Dieu de Paris, France.

A case of disseminated superficial porokeratosis (DSP) is reported in a black man 5 years after
autologous bone marrow transplantation (BMT) for acute promyelocytic leukemia. Porokeratosis
is a rare hyperkeratotic disorder arising from clonal keratinocytes with a high potential to develop
squamous cell carcinoma. Inherited forms are classical but recent observations of acquired
porokeratosis have been reported in immunocompromized patients (AIDS, immune disorders,
immune suppressive drugs or organ transplantation). Two cases of DSP have been reported after
allogeneic BMT in patients treated for chronic GVHD. Our case is the first one after autologous
BMT, in a black man, on no immunosuppressive drug at the time of diagnosis of DSP.
Hematopoietic and immune reconstitution was apparently complete. The cancer-prone character of
porokeratosis could be favored by total body irradiation used in conditioning regimen. Thus,
porokeratosis has to be associated with other late effects after BMT such as HCV seropositivity,
cataract and infertility that were observed in this patient. 

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7.) Cancer proneness of linear porokeratosis may be explained by allelic loss.
========================================================================
Dermatology 1997;195(1):20-5 (ISSN: 1018-8665)

Happle R [Find other articles with this Author]

Department of Dermatology, Philipp University of Marburg, Germany.
[email protected].

BACKGROUND: It is well known that porokeratosis, a genetically heterogeneous disorder
characterized by the histopathological feature of the cornoid lamella, shows an increased proneness
to develop carcinoma. On the other hand, a significant mechanism in the origin of many forms of
cancer is loss of heterozygosity or allelic loss. OBJECTIVE: Because it has recently been proposed
that linear porokeratosis may result from allelic loss, one might expect that linear porokeratosis is
especially prone to malignant degeneration. In order to test this hypothesis, a review of case reports
was performed. METHOD: Cases of cancer-associated porokeratosis were collected from the
European language literature and assigned to one of 5 different types [plaque type of Mibelli (PM);
disseminated actinic superficial porokeratosis (DSAP); porokeratosis palmaris, plantaris et
disseminata (PPPD); porokeratosis punctata palmaris et plantaris (PPPP); linear porokeratosis
(LP)]. RESULTS: Malignant or premalignant lesions were reported in 9 cases of PM, 15 cases of
DSAP, 3 cases of PPPD, 1 case of PPPP and 21 cases of LP. CONCLUSION: This analysis
supports the view that among the various forms of porokeratosis, the linear type is particularly
susceptible to malignant degeneration. Arguments are presented in favor of the assumption that the
genetic mechanism of allelic loss giving rise to LP may represent an initial step in the development of
cancer. 

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8.) Porokeratotic palmoplantar keratoderma discreta--a new entity or a variant of porokeratosis plantaris discreta?
========================================================================
Clin Exp Dermatol 1996 Nov;21(6):451-3 (ISSN: 0307-6938)

Korstanje MJ; Vrints LW [Find other articles with these Authors]

Department of Dermatology, St Anna Hospital, Geldrop, The Netherlands.

We report a family with hyperkeratotic lesions on palms and soles. The lesions became evident in
the second to third decade, and there is an autosomal dominant mode of transmission. Skin biopsy
specimens show a central epidermal depression filled by a compact hyperkeratotic plug of columnar
parakeratosis, like a broad cornoid lamella. The lesions resemble porokeratosis plantaris discreta
clinically and histologically. The cornoid lamella is a broad, solid keratin plug rather than a
centrifugally enlarging annular or serpentine ridge as can been seen in other types of porokeratosis.
Perhaps the lesions of porokeratosis plantaris discreta should not be classified as a true
porokeratosis but as porokeratotic plantar keratoderma discreta. We have therefore called the
lesions in our patients porokeratotic palmoplantar keratoderma discreta, and suggest that
porokeratotic palmoplantar keratoderma discreta is a variant of porokeratosis plantaris discreta. 

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9.) Disseminated superficial porokeratosis developing after electron-beam total skin irradiation for mycosis fungoides.
========================================================================
Clin Exp Dermatol 1996 Jul;21(4):310-2 (ISSN: 0307-6938)

Romani J; Pujol RM; Casanova JM; de Moragas JM [Find other articles with these Authors]

Hospital de la Santa Creu i Sant Pau, Spain.

A 74-year-old man with stage IB cutaneous T-cell lymphoma was treated with electron-beam total
skin irradiation in 1988. Seven years later, multiple disseminated lesions of porokeratosis
developed on the chest, extremities and abdomen. There was no family history of porokeratosis,
nor history of treatment with PUVA or of excessive sun exposure. Development of disseminated
porokeratosis on nonexposed sites suggests a direct role for the previous ionizing radiation.
Electron-beam total skin irradiation therapy should therefore be added to the list of possible
causative factors in the development of disseminated porokeratosis. 

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10.) Disseminated superficial porokeratosis with amyloid deposition.
========================================================================
J Dermatol 1996 Feb;23(2):111-5 (ISSN: 0385-2407)

Yasuda K; Ikeda M; Ikeda M; Kodama H [Find other articles with these Authors]

Department of Dermatology, Kochi Medical School, Japan.

Two patients with disseminated superficial porokeratosis (DSP) with amyloid deposition are
reported. The skin lesions were distributed over both sun-exposed areas and sun-protected areas.
No exacerbation by sun exposure was noted. Abundant amyloid substances were deposited at the
papillary dermis, not only beneath the cornoid lamellae but also within the annular margin. The skin
lesions and amyloid deposition regressed with topical application of dimethyl sulfoxide in one
patient, suggesting that DSP is one of the clinical phenotypes of primary localized cutaneous
amyloidosis. Another possibility is that amyloid substances are produced by poorly differentiated
keratinocytes in DSP. 

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11.) Homozygote erythropoietic protoporphyria associated with porokeratosis]
[Protoporphyrie erythropoietique homozygote associee a une porokeratose.]
========================================================================
Ann Dermatol Venereol 1996;123(6-7):382-6 (ISSN: 0151-9638)

Philippot V; Berard F; Perrot H [Find other articles with these Authors]

Service de Dermatologie, Hopital de l'Hotel-Dieu, Lyon.

INTRODUCTION: Erythropoietic protoporphyria was generally assumed to be an autosomal
dominant disease with variable penetrance. The determination of the ferrochelatase activity and the
biological molecular studies have shown that both autosomal dominant and recessive patterns of
inheritance are possible. CASE REPORT: Is reported the case of a 17 years-old male patient with
erythropoietic protoporphyria and porokeratosis. There are some hepatic biochemical
abnormalities without cholelithiasia and without pathological change of the liver biopsy. Leucocyte
ferrochelatase activity is decreased to 5 p. 100 of the normal mean level. In both the parents,
without photosensitivity, the enzyme activity is reduced to 40 p. 100 of the normal values.
DISCUSSION: The patients with severe ferrochelatase defect have no more important clinical
manifestations than in the usual form of erythropoietic protoporphyria. For clarify the exact mode of
inheritance, the determination of the ferrochelatase activity and the identification of the mutations in
the patient and his parents are necessary. In our patient the porokeratosis should be in relation
with the protoporphyrin induced phototoxic reaction which facilitate the emergence of a mutant
cellular clone of epithelial cells. 

========================================================================
12.) [Mibelli porokeratosis in 2 brothers]
[Porokeratose de Mibelli chez deux freres.]
========================================================================
Ann Dermatol Venereol 1996;123(3):171-3 (ISSN: 0151-9638)

Yedomon HG; Do Ango-Padonou F [Find other articles with these Authors]

Service de Dermatologie, Centre National Hospitalier et Universitaire (CNHU), Cotonou Benin.

INTRODUCTION: Mibelli's porokeratosis is uncommon in black persons. We report two
brothers who had two different clinical presentations. CASE REPORT: The brothers were seen at
the ages of 16 and 19 years. Both had Mibelli's porokeratosis, one with a papulo-verruciform
presentation located on the scrotum, the anus, the gluteal area and the back of the hand, and the
other with a superficial disseminated eruption involving the face and the forearm. DISCUSSION:
The incidence of Mibelli's porokeratosis in the black population at Cotonou is approximately 0.3
per 10.000. The presence of the disease in two uterine brothers confirms the monogenic and familial
nature of Mibelli's porokeratosis. Dominant transmission cannot be easily demonstrated when the
parents of the patients are phenotypically healthy. 

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13.) Linear porokeratosis presenting as erosions in the newborn period.
========================================================================
Pediatr Dermatol 1995 Dec;12(4):318-22 (ISSN: 0736-8046)

Fisher CA; Le Boit PE; Frieden IJ [Find other articles with these Authors]

Dermatology Department, National Naval Medical Center, Bethesda, Maryland, USA.

The classic appearance of porokeratosis is characterized by a hyperkeratotic annular rim that
expands peripherally, leaving an atrophic center. Linear porokeratosis is a variant with collections
of such lesions arranged in a linear fashion, usually corresponding to a dermatome or Blaschko's
lines. Ulcerations have rarely been reported in patients with porokeratosis. We report an unusual
case of linear porokeratosis at birth, with erosions and ulcerations of the face and lower extremity,
that eluded diagnosis for nearly a year. Porokeratosis should be considered in the differential
diagnosis of erosions in the newborn period. 

========================================================================
14.) Genital porokeratosis of Mibelli.
========================================================================
Genitourin Med 1995 Dec;71(6):410-1 (ISSN: 0266-4348)

Neri I; Marzaduri S; Passarini B; Patrizi A [Find other articles with these Authors]

Department of Dermatology, University of Bologna, Italy.

Porokeratosis of Mibelli is a disorder of epidermal proliferation in which many different clinical
forms can be distinguished. Two male patients with a localized type of porokeratosis limited to the
genitalia are reported. Later in life they developed an annular skin lesion with peripheral keratotic
ridge. The histological examination of a biopsy specimen showed the characteristic features of
porokeratosis. There was no family history of similar skin disorders and the patients were not on
any drugs. Genital porokeratosis is probably underdiagnosed and we believe that these patients
should be followed up on account of the precancerous potential of this disease. 

========================================================================
15.) Disseminated epidermolytic acanthoma with disseminated superficial porokeratosis and verruca vulgaris in an immunosuppressed patient.
========================================================================
J Dermatol 1995 Sep;22(9):690-2 (ISSN: 0385-2407)

Chun SI; Lee JS; Kim NS; Park KD [Find other articles with these Authors]

CHA General Hospital, Department of Dermatology, Yonsei University College of Medicine,
Seoul, Korea.

A 40-year-old man who had received long term immunosuppressive treatment for 14 years
following kidney transplantation developed multiple skin lesions on both antecubital fossae, scalp,
and both lower extremities. Histopathologic findings from three skin regions revealed characteristic
features of epidermolytic hyperkeratosis, verruca vulgaris, and disseminated superficial
porokeratosis, respectively. Although immunocompromised individuals may demonstrate verruca
vulgaris or porokeratosis, disseminated epidermolytic acanthoma (DEA) has not been reported to
be associated with immunosuppressed status. We suggest that immunosuppression may play a role
in the pathogenesis of DEA, as shown in our case. 

========================================================================
16.) Rapid development of disseminated superficial porokeratosis after transplant induction therapy.
========================================================================
Bone Marrow Transplant 1995 Jun;15(6):993-5 (ISSN: 0268-3369)

Fields LL; White CR Jr; Maziarz RT [Find other articles with these Authors]

Department of Dermatology, Oregon Health Sciences University, Portland, USA.

Disseminated superficial actinic porokeratosis (DSAP) is a cutaneous disorder, usually inherited in
an autosomal dominant fashion, characterized by numerous annular papules with subtle raised
hyperkeratotic borders and slightly atrophic centers. While the precise pathophysiologic
mechanisms underlying the development of DSAP are unknown, one hypothesis is multifocal
expansion of atypical clones of keratinocytes, perhaps unmasked by actinic damage, as implied by
its name. Although primarily of cosmetic concern, there is an increased risk of squamous cell
carcinoma of the skin developing within DSAP lesions, which often show histologic keratinocytic
atypia centrally. Immunosuppression, which is a significant risk factor for secondary malignancies
including cutaneous squamous cell carcinoma, is also a well-documented precipitant of
porokeratosis. We report a 62-year-old man who developed DSAP in a widely and rapidly
progressive manner within days of receiving total body radiation and high-dose induction
chemotherapy as planned preparative therapy for an autologous peripheral blood stem cell
transplant for relapsed high grade lymphoma. Our patient's eruption of DSAP highlights a little
recognized cutaneous manifestation of aggressive bone marrow transplant induction therapy. 

========================================================================
17.) Porokeratosis and Crohn's disease.
========================================================================
J Am Acad Dermatol 1995 May;32(5 Pt 2):894-7 (ISSN: 0190-9622)

Morton CA; Shuttleworth D; Douglas WS [Find other articles with these Authors]

Department of Dermatology, Monklands District General Hospital, Airdrie, Scotland.

We report the cases of three patients with Crohn's disease in whom porokeratosis developed.
Disseminated superficial actinic porokeratosis developed in two patients. In one of these patients,
the skin lesions arose during an exacerbation of the bowel disease. In the third patient, who had
congenital linear porokeratosis, the disseminated superficial form of the disorder developed during
the first severe exacerbation of Crohn's disease. A family history of porokeratosis was present in
one patient, but no relatives of any of these patients were known to have Crohn's disease. In all
three patients, Crohn's disease was limited to the colon. 

========================================================================
18.) Porokeratosis and immunosuppression.
========================================================================
SO - Br J Dermatol 1995 Jan;132(1):74-8
AU - Bencini PL; Tarantino A; Grimalt R; Ponticelli C; Caputo R
AD - Istituto di Clinica Dermatologica I e Dermatologia Pediatrica Universita di Milano, Ospedale Maggiore IRCCS, Italy.
PT - JOURNAL ARTICLE
AB - Immunosuppression may favour the development of disseminated superficial porokeratosis (DSP). We report the clinical features and the outcome of DSP in 24 patients receiving immunosuppressive treatment (group A), and compare the characteristics of the disease with those of 13 immunocompetent patients with DSP (group B). The two groups were similar with regard to age, sex, area of skin involvement and mean follow-up. There was a family history of DSP in only two patients in group A, compared with five patients in group B (P = 0.03). The skin type, based on the tanning response to sunlight, was not significantly different between the two groups. Two of the 24 patients in group A had high sun exposure, compared with five of the 13 patients in group B (P = 0.03). Moreover, 10 patients in group A and 11 in group B (P = 0.01) exhibited worsening of the disease after exposure to sunlight, usually during the summertime. These observations appear to support the hypothesis that sun exposure is not always essential for the development of porokeratosis in immunosuppressed patients. None of our patients developed skin cancer in porokeratotic lesions during the follow-up period.

========================================================================
19.) Porokeratosis of Mibelli associated with active chronic hepatitis and vitiligo.
========================================================================
SO - Acta Derm Venereol 1994 Nov;74(6):463-4
AU - Dippel E; Haas N; Czarnetzki BM
AD - Department of Dermatology, Clinics Rudolf Virchow, Free University Berlin, Germany.
PT - JOURNAL ARTICLE
AB - A patient with porokeratosis Mibelli is reported who suffered from long-standing chronic active hepatitis and rapidly expanding vitiligo of more recent onset. This type of disease association has never been reported before, although it is in line with several reports of porokeratosis in association with immunoregulatory disorders, mostly after drug-induced immunosuppression. The lesions of the present patient responded well to treatment with topical 5-fluorouracil.

========================================================================
20.) Disseminated superficial actinic porokeratosis: experimental induction and exacerbation of skin lesions.
========================================================================
SO - J Am Acad Dermatol 1989 Dec;21(6):1182-8
AU - Neumann RA; Knobler RM; Jurecka W; Gebhart W
AD - Department of Dermatology II, University of Vienna, Austria.
PT - JOURNAL ARTICLE
AB - A 55-year-old woman with disseminated superficial actinic porokeratosis (DSAP) had lesions on sun-exposed skin areas that were exacerbated during the summer months and involuted in winter. This is the third report in which induction and exacerbation of DSAP lesions were achieved by irradiation with artificial ultraviolet light sources. Our data show that UVB plus UVA is more effective in inducing new or exacerbating preexisting skin lesions than either wavelength alone. We believe that testing with the appropriate ultraviolet light sources is a practical means to differentiate between DSAP and disseminated superficial porokeratosis.

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21.) Disseminated superficial porokeratosis in a patient with AIDS.
========================================================================
SO - Br J Dermatol 1994 Aug;131(2):284-9
AU - Kanitakis J; Misery L; Nicolas JF; Lyonnet S; Chouvet B; Haftek M; Faure M; Claudy A; Thivolet J
AD - Department of Dermatology, Hopital Edouard Herriot, Lyon, France.
PT - JOURNAL ARTICLE
AB - We report the case of a 50-year-old male homosexual suffering from AIDS, who developed diffuse annular hyperkeratotic lesions on the arms and legs. Histopathological examination revealed typical features of porokeratosis, which clinically was of the disseminated superficial type. Ultrastructural examination showed a paucity of keratohyalin granules and lamellar bodies. Immunohistochemical studies showed an almost complete absence of Langerhans cells in lesional epidermis. Involucrin and filaggrin expression were altered in areas of cornoid lamella formation, whereas basal keratinocytes in these areas expressed PCNA/cyclin and, to a lesser degree, p53 protein. Porokeratosis may affect immunocompetent patients, but has also been reported in the setting of immunosuppression following organ transplantation. As far as we are aware, the development of porokeratosis during the course of HIV infection has not been reported previously.

========================================================================
22.) Elevated chromosome aberration frequency after X-ray exposure of cultured fibroblasts derived from patients with porokeratosis.
========================================================================
SO - Cancer Genet Cytogenet 1994 Apr;73(2):161-4
AU - Takeshita T; Higurashi M; Ariizumi-Shibusawa C; Shimizu K; Iijima S; Yamagata Z; Asaka A; Morimoto K; Ishibashi Y; Otsuka F
AD - Department of Health Sciences, Yamanashi Medical University, Japan.
PT - JOURNAL ARTICLE
AB - Porokeratosis (PK) is a rare genetic skin disorder inherited as an autosomal dominant trait and regarded as a disease predisposing to cancer. To evaluate chromosomal radiosensitivity of PK cells, we examined chromosome aberration frequency after X-irradiation of cultured skin fibroblasts derived from PK patients and controls. Without X-ray exposure, frequencies of chromosome-type aberrations (exchanges or deletions) were not different between the patients and controls. Following X-ray irradiation, frequencies of deletions in the patient group were significantly increased, whereas those of exchanges were not elevated. No differences in chromatid-type aberration frequency were found between the patients and controls with or without exposure to X-ray. The observed radiosensitivity, though not as high as in ataxia telangiectasia (AT) cells, agrees well with the previously reported higher radiosensitivity of PK fibroblasts in survival analysis.

========================================================================
23.) Overexpression of p53 tumor suppressor protein in porokeratosis [see comments]
========================================================================
CM - Comment in: Arch Dermatol 1995 Mar; 131(3):353-4
SO - Arch Dermatol 1994 Feb;130(2):187-90
AU - Magee JW; McCalmont TH; Le Boit PE
AD - Department of Pathology, University of California, San Francisco.
PT - JOURNAL ARTICLE
AB - BACKGROUND: p53 is a tumor suppressor nucleoprotein. Mutations of the p53 gene have been found in a variety of malignant neoplasms. Wild-type p53 has a short half-life, possibly only 20 to 30 minutes, and is not present in the nucleus at levels that are detectable with routine immunohistochemical techniques. Mutant p53 has a longer half-life, and is readily detectable with immunoperoxidase staining. RESULTS: We studied 17 specimens from patients with either porokeratosis of Mibelli or actinic porokeratosis, using immunoperoxidase staining with an antibody directed against the p53. There was staining of lesional keratinocyte nuclei in 16 of 17 specimens, limited in most cases to the zone between cornoid lamellae. Staining for proliferating cell nuclear antigen was increased above background levels in only six of 13 specimens. CONCLUSIONS: The finding of p53 immunoperoxidase staining in porokeratosis suggests genetic mutation, as occurs in other cutaneous keratinocytic neoplasms, and the lack of corresponding proliferating cell nuclear antigen expression in many specimens indicates that p53 overexpression is not simply a reflection of increased cellular proliferation.

========================================================================
24.) [Squamous cell carcinoma and disseminated superficial actinic porokeratosis]
========================================================================
TT - [Carcinome spinocellulaire et porokeratose actinique superficielle disseminee.]
SO - Ann Dermatol Venereol 1994;121(1):50-2
AU - Remond B; Dompmartin A; Mandard JC; Leroy D
AD - Service de Dermatologie, CHU, Caen.
MC - English Abstract
PT - JOURNAL ARTICLE
AB - Disseminated superficial actinic porokeratosis was first described by Chernosky and Anderson in 1969. It is characterized by multiple small keratotic lesions on sun-exposed areas beginning in the third of fourth decade. The development of a squamous cell carcinoma within lesions of porokeratosis or the association of superficial actinic porokeratosis with immunosuppression have been well documented. We report the case of a 68-year-old patient who presented actinic porokeratosis associated with rapidly evolutive squamous cell carcinoma of the leg. During the hospitalization, an IgA myeloma was discovered. The authors discuss the relationship between porokeratosis, immunosuppression, and squamous cell carcinoma. Pathogenesis of the lesions is interesting because it is admitted that a keratinocyte clone which carries the porokeratosis abnormality is going to proliferate because of immunosuppression, trauma and infectious diseases. It seems important to search for immunosuppression in patients presenting porokeratosis because the incidence of malignant transformation may increase.

========================================================================
25.) The coexistence of linear and giant porokeratosis associated with Bowen's disease.
========================================================================
SO - Dermatology 1994;189(1):78-80
AU - Lucker GP; Steijlen PM
AD - Department of Dermatology, University Hospital, Nijmegen, The Netherlands.
PT - JOURNAL ARTICLE
AB - Linear and giant porokeratosis are both rare variants of this disorder of keratinization. We present a case exhibiting the clinical features of both variants coexisting in one patient, which to our knowledge has not been described previously. Furthermore, Bowen's disease was found to be present in the giant lesion, reflecting the enhanced risk of malignant transformation of this rare subtype of porokeratosis.

========================================================================
26.) Porokeratosis in immunosuppressed and nonimmunosuppressed patients.
========================================================================
SO - Int J Dermatol 1992 Nov;31(11):781-2
AU - Raychaudhuri SP; Smoller BR
AD - Department of Dermatology and Pathology, Stanford University School of Medicine, California.
PT - JOURNAL ARTICLE
AB - Porokeratosis is an uncommon, inherited, autosomally dominant disorder. In the last decade association of porokeratosis and immunosuppression has been observed. In this study we carried out a comparative study between immunosuppressed and nonimmunosuppressed porokeratosis cases. We found that 9 out of 20 cases of porokeratosis were associated with organ transplantation/immunosuppression. Clinicopathologic study revealed that the pattern of disease is alike both in immunosuppressed and nonimmunosuppressed patients. Our observations indicate that immune modulation could be a factor in the genesis of porokeratosis.

========================================================================
27.) Eruptive pruritic papular porokeratosis.
========================================================================
SO - J Dermatol 1992 Feb;19(2):109-12
AU - Kanzaki T; Miwa N; Kobayashi T; Ogawa S
AD - Department of Dermatology, Nagoya City University Medical School, Japan.
PT - JOURNAL ARTICLE
AB - Three cases of an unusual variant of porokeratosis (Mibelli) were described. Patients with disseminated superficial porokeratosis for some years suddenly developed intensively pruritic erythematous papules. Skin biopsies revealed that these papules contained cornoid lamellae on their tops. Pruritic papules subsided in several months, leaving slightly hyperkeratotic brown annular lesions which were shown to contain typical cornoid lamellae histopathologically. This type of porokeratosis has not been reported in the literature.

========================================================================
28.) Porokeratosis palmaris et plantaris disseminata. Report of a case with abnormal DNA ploidy in lesional epidermis.
========================================================================
SO - Arch Dermatol 1992 Feb;128(2):236-9
AU - Beers B; Jaszcz W; Sheetz K; Hogan DJ; Lynch PJ
AD - Department of Dermatology, University of Minnesota Hospital, Minneapolis.
PT - JOURNAL ARTICLE
AB - BACKGROUND--Porokeratosis is believed to be a premalignant condition of the skin. Recent flow cytometric studies showing DNA aneuploidy in the epidermis of some types of porokeratosis support this conclusion. We describe a patient with porokeratosis palmaris et plantaris disseminata in whom abnormal DNA ploidy was found in lesional epidermis with the use of flow cytometry. OBSERVATIONS--DNA flow cytometry of lesional epidermis from the back showed two populations of cells, one with diploid and the other with aneuploid DNA content and DNA index of 1.1 (hyperdiploid). The coefficient of variation of the diploid and aneuploid peaks was 2.1% and 4.1%, respectively. CONCLUSIONS--Porokeratosis palmaris et plantaris disseminata, like other forms of porokeratosis, exhibits abnormal DNA ploidy in lesional epidermis. This finding underscores the premalignant nature of this and other forms of porokeratosis.

========================================================================
29.) Porokeratosis of Mibelli following heart transplant [see comments]
========================================================================
CM - Comment in: Int J Dermatol 1992 Sep; 31(9):673
SO - Int J Dermatol 1992 Jan;31(1):52-4
AU - Rothman IL; Wirth PB; Klaus MV
AD - Department of Dermatology, School of Medicine and Health Sciences, State University of New York, Buffalo 14214.
PT - JOURNAL ARTICLE; REVIEW (23 references); REVIEW OF REPORTED CASES
AB - A 59-year-old white man with a history of Sydenham's chorea received a mitral valve prosthesis in 1962. He sustained an anterolateral myocardial infarction in 1983. In 1984, he received a heart transplant. To prevent heart rejection, he was initially treated with cyclosporine 12 mg/kg/day and prednisone 90 mg b.i.d. In 1987, azathioprine 100 mg daily was added. In 1989, at the time of our evaluation, his medications included cyclosporine 80 mg b.i.d., prednisone 10 mg b.i.d., and azathioprine 75 mg/day. Since his heart transplant surgery he had not taken any thiazide medication. The patient noted a lesion on his right thigh; the lesion appeared in 1986, 2 years after his heart transplant. On examination in 1989, the lesion was a 2 cm wide annular plaque with a shiny atrophic center and raised border. Both the clinical appearance and pathology were consistent with a diagnosis of porokeratosis of Mibelli. No family history of porokeratosis was elicited.

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30.) Linear and punctate porokeratosis associated with end-stage liver disease.
========================================================================
SO - J Am Acad Dermatol 1991 Nov;25(5 Pt 2):937-9
AU - Hunt SJ; Sharra WG; Abell E
AD - Department of Dermatology, University of Pittsburgh, Pennsylvania.
PT - JOURNAL ARTICLE
AB - A periodic eruption of porokeratosis developed in a 31-year-old black woman with chronic idiopathic hepatitis requiring liver transplantation. The clinicopathologic features were chiefly those of linear and punctate porokeratosis but overlapped those of porokeratosis plantaris, palmaris et disseminata and hyperkeratotic or verrucous porokeratosis. Typical cornoid lamellae were visible on histologic examination. Outbreaks of the lesions occurred during exacerbations of the liver disease. The skin condition rapidly improved after operation, with concomitant improvement in liver function.

========================================================================
31.) Morphogenesis of the cornoid lamella: histochemical, immunohistochemical, and ultrastructural study of porokeratosis.
========================================================================
SO - J Cutan Pathol 1991 Aug;18(4):247-56
AU - Ito M; Fujiwara H; Maruyama T; Oguro K; Ishihara O; Sato Y
AD - Department of Dermatology, Niigata University School of Medicine, Japan.
PT - JOURNAL ARTICLE
AB - To elucidate the morphogenesis of cornoid lamellae (CL) in porokeratosis, skin lesions of three cases of disseminated superficial actinic porokeratosis and a case of linear porokeratosis were examined. By N-(7-dimethylamino-4-methyl-3-coumarinyl)maleimide staining, SH groups were present in the living layer of the epidermis beneath CL and irregularly disappeared at the bottom of CL, whereas SS linkages appeared in dyskeratotic cells in the living layer and in the irregularly shaped cell membranes of the horny cells. Epidermis beneath CL showed an increased and irregular involucrin expression. Ultrastructurally, the living keratinocytes contained many cytoplasmic vacuoles and had a smaller number of lamellar bodies than normal. Intercellular lamellar sheets were incompletely formed. The dyskeratotic cells and the lower horny cells contained many small vacuoles but formed a marginal band. The horny cells of CL also formed a marginal band and, further, a keratin pattern. CL may be formed by hyperproliferative atypical kertatinocytes which keratinize rapidly and irregularly and show defective desquamation due to the paucity of intercellular lamellar sheets.

========================================================================
32.) Porokeratosis arising in a burn scar.
========================================================================
SO - J Am Acad Dermatol 1991 Aug;25(2 Pt 2):354-6
AU - Nova MP; Goldberg LJ; Mattison T; Halperin A
AD - Dermatopathology Associates of New York, Albert Einstein College of Medicine.
PT - JOURNAL ARTICLE; REVIEW (21 references); REVIEW OF REPORTED CASES
AB - A histologically verified porokeratosis arose in a burn scar on the back of a 47-year-old man. This phenomenon has not been previously reported. We discuss the literature on porokeratosis, the relation between epidermal and dermal injury, and the genesis of this lesion.

========================================================================
33.) Disseminated superficial actinic porokeratosis. A histological review of 61 cases ========================================================================
with particular reference to lymphocytic inflammation.
SO - Am J Dermatopathol 1991 Feb;13(1):26-31
AU - Shumack S; Commens C; Kossard S
AD - Skin and Cancer Foundation, Darlinghurst, N.S.W., Australia.
PT - JOURNAL ARTICLE
AB - The pathology of 61 cases of disseminated superficial actinic porokeratosis was reviewed and the relative frequency of the histological features associated with the cornoid lamella and the pathology within and outside the porokeratotic rim were assessed. Papillary dermal lymphocytic infiltrate (97%), spinous layer vacuolar changes (90%), dyskeratotic cells in the epidermis (77%), and liquefaction degeneration of the basal layer (67%) were frequently seen under the cornoid lamella. Papillary lymphocytic infiltration was seen more frequently inside the porokeratotic ring in comparison to the outer skin. Lymphocyte marker studies in nine cases showed a predominance of activated T lymphocytes with positive LN3 and UCHL-1 staining. Together with the finding of a lichenoid reaction pattern, these results lend support to the hypothesis that actinic porokeratosis represents a migrating clone of abnormal keratinocytes with an associated immunological host response.

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34.) Superficial actinic porokeratosis and cystic fibrosis.
========================================================================
SO - Acta Derm Venereol 1991;71(5):440-1
AU - Klapholz L; Goldenhersh M; Sherman Y; Leibovici V
AD - Department of Dermatology, Hadassah University Hospital, Jerusalem, Israel.
PT - JOURNAL ARTICLE
AB - A 24-year-old woman, presenting with cystic fibrosis, developed superficial actinic porokeratosis. Immunosuppression due to cystic fibrosis may be either the cause of or the exacerbating factor in superficial actinic porokeratosis in our patient.

========================================================================
35.) [Linear porokeratosis of Mibelli in monozygotic twin girls]
========================================================================
TT - [Porokeratose de Mibelli lineaire chez des jumelles monozygotes.]
SO - Ann Dermatol Venereol 1991;118(8):519-24
AU - Guillot P; Taieb A; Fontan I; Bilhou-Nabera C; Viard E; Renaud P; Maleville J
AD - Service de Dermatologie, Hopital des Enfants, Bordeaux.
MC - English Abstract
PT - JOURNAL ARTICLE; REVIEW (37 references); REVIEW OF REPORTED CASES
AB - Two monozygotic female twins with linear porokeratosis of Mibelli are described. One had only minimal lesions of the right elbow. The other had a linear lesion of the right arm following Blaschko's lines. Monozygotism was established using DNA fingerprinting. A single-gene defect of variable expressivity involving the clonal development of epidermal cutaneous cells according to the lines of cutaneous embryogenesis is suspected. Dithranol and carbon dioxide laser treatment are discussed.

========================================================================
36.) [Mibelli's porokeratosis gigantea]
========================================================================
TT - [Porokeratosis Mibelli gigantea.]
SO - Hautarzt 1990 Nov;41(11):633-5
AU - Bacharach-Buhles M; Weindorf N; Altmeyer P
AD - Dermatologische Klinik, Ruhr-Universitat Bochum.
MC - English Abstract
PT - JOURNAL ARTICLE
AB - This case report deals with the giant form of porokeratosis of Mibelli, porokeratosis Mibelli gigantea. Over the last 40 years, the 55-year-old man concerned had observed a centrifugal expansion of a single hyperkeratotic lesion until it covered the whole of the right hand and forearm. Besides the characteristic histological alterations, we saw capillaries with unusually thickened walls owing to basal lamina multiplication. In the centre of the lesion atrophy of the epidermis and of the corium was visible. These observations indicate an alteration in two germ layers in porokeratosis.

========================================================================
37.) Disseminated porokeratosis in an infant with craniosynostosis.
========================================================================
SO - Br J Dermatol 1990 Aug;123(2):249-54
AU - Judge MR; Michaels M; Sams VR; David TJ; Harper JI
AD - Hospitals for Sick Children, London, U.K.
PT - JOURNAL ARTICLE
AB - An infant with craniosynostosis and other congenital defects developed a progressive skin rash from the age of 1 month. Histological examination revealed dyskeratosis and a cornoid lamella suggestive of porokeratosis. This patient is remarkable for the early onset and severity of the skin disease.

========================================================================
38.) Disseminated porokeratosis accompanying multicentric Bowen's disease. ========================================================================
Characterization of porokeratotic lesions progressing to Bowen's disease.
SO - J Am Acad Dermatol 1990 Aug;23(2 Pt 2):355-9
AU - Otsuka F; Huang J; Sawara K; Asahina A; Ishibashi Y
AD - Department of Dermatology, Tokyo University Hospital, Japan.
PT - JOURNAL ARTICLE
AB - Multicentric Bowen's disease developed in a 73-year-old man in areas involved with disseminated porokeratosis. The porokeratotic lesions were brownish; some were small and solitary, and others were coalesced and of variable size. Some lesions were erythematous. The histologic findings of the small, solitary or brownish, coalesced lesions were those of porokeratosis without apparent dysplasia; however, the erythematous coalesced lesions revealed epidermal dysplasia. Study revealed DNA ploidy abnormalities in the epidermal cells of these porokeratotic skin lesions. The skin lesions progressed in their degree of DNA ploidy abnormality from small solitary lesions to brownish coalesced ones and further to erythematous, coalesced ones. These observations suggest the direct and sequential growth of potentially malignant neoplastic clones in the patient's porokeratotic skin lesions and explain the multicentric development of Bowen's disease.

========================================================================
39.) Unusual presentation of porokeratosis palmaris, plantaris et disseminata.
========================================================================
SO - J Am Acad Dermatol 1989 Nov;21(5 Pt 2):1131-3
AU - Neumann RA; Knobler RM; Gebhart W
AD - Department of Dermatology II, University of Vienna, Austria.
PT - JOURNAL ARTICLE
AB - Porokeratosis plantaris, palmaris et disseminata is an autosomal dominant genodermatosis characterized by multiple lesions on the palms and soles, and later on other areas, both sun-exposed and non-sun-exposed. We report a 66-year-old man with porokeratosis plantaris, palmaris et disseminata whose disease had an unusual evolution. To our knowledge this is the first case of the disease in which the lesions first appeared on the trunk and extremities and later involved the palms and soles.

========================================================================
40.) Clonal chromosome abnormalities with preferential involvement of chromosome 3 in patients with porokeratosis of Mibelli.
========================================================================
SO - Cancer Genet Cytogenet 1989 Nov;43(1):89-94
AU - Scappaticci S; Lambiase S; Orecchia G; Fraccaro M
AD - Biologia Generale e Genetica Medica Universita di Pavia, Italy.
PT - JOURNAL ARTICLE
AB - Clonal chromosome abnormalities were found in cultured fibroblasts from three sibs and one sporadic case with porokeratosis of Mibelli. Chromosome 3 especially involved region p12-14. This region includes the most common fragile site in humans, and the proximal region of chromosome 3 short arm is involved in a variety of neoplastic conditions. We conclude that porokeratosis of Mibelli, an autosomal dominant disorder, is associated with chromosomal instability. Porokeratosis of Mibelli is known to also be associated with increased susceptibility to malignant disease. The chromosome instability may well predispose to malignancy.

========================================================================
41.) Zosteriform porokeratosis: a report of two cases.
========================================================================
SO - Cutis 1989 Sep;44(3):216-9
AU - Veraldi S; Bocor M; Gasparini G
AD - First Department of Dermatology and Pediatric Dermatology, University of Milan, Italy.
PT - JOURNAL ARTICLE
AB - The authors describe two cases of zosteriform porokeratosis occurring in two female patients, aged seventeen and fourteen years. In both patients the dermatosis was localized to the thigh; one patient also presented with linear lesions in the thoracoabdominal and dorsal regions. Histopathologic examination confirmed the clinical diagnosis and excluded the presence of atypical cells.

========================================================================
42.) Porokeratosis punctata palmaris et plantaris. A new entity?
========================================================================
SO - Arch Dermatol 1989 Jun;125(6):816-9
AU - Lestringant GG; Berge T
AD - Department of Dermatology, Al-Hada Military Hospital, Taif, Kingdom of Saudi Arabia.
PT - JOURNAL ARTICLE
AB - Seven members from three generations of a Saudi family presented with porokeratosis punctata palmaris et plantaris (PPPP). In our series of patients, the disorder began in their early 20s, seemed to be transmitted as a dominant trait, and affected males only. The elementary lesion was a tiny keratotic spine, and the involvement was strictly limited to the volar aspects of the hands and to the soles of the feet. Histological studies showed a columnar parakeratosis that resembled the cornoid lamella of porokeratosis, but other clinical and histological traits tended to make PPPP an entity that was distinct from true porokeratosis. There have been only six reports in the literature on genuine PPPP before this series.

========================================================================
43.) Porokeratosis as a premalignant condition of the skin. Cytologic demonstration of abnormal DNA ploidy in cells of the epidermis.
========================================================================
SO - Cancer 1989 Mar 1;63(5):891-6
AU - Otsuka F; Shima A; Ishibashi Y
AD - Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
PT - JOURNAL ARTICLE
AB - Clinical evidence has accumulated of malignant epithelial tumors developing on the lesions of porokeratosis. To determine the cytologic basis for the malignant change of porokeratosis, the nuclear DNA content of epidermal cells from porokeratotic lesions was measured using microfluorometry. A total of 42% of 33 porokeratotic skin lesions in eight of the 16 patients showed DNA polyploidization in the epidermis. Most of the porokeratotic skin lesions, with or without DNA polyploidization, increase cell proportions in the S and G2/M phase range. DNA indices of cells from these porokeratotic lesions distributed widely from the level of normal control epidermis to that of malignant epidermal conditions. These findings suggest that porokeratosis is undergoing the neoplastic process, and is a precursor of malignant tumors.

========================================================================
44.) Porokeratosis of mibelli in transplant recipients.
========================================================================
SO - Am J Clin Pathol 1989 Jan;91(1):71-4
AU - Komorowski RA; Clowry LJ
AD - Department of Pathology, Medical College of Wisconsin, Milwaukee 53226.
PT - JOURNAL ARTICLE
AB - Four of 602 renal and hepatic transplant recipients had porokeratosis of Mibelli develop in the posttransplant period. Porokeratosis is an uncommon, autosomally dominant inherited disorder that presents in adolescence as a proliferation of an abnormal clone of epidermal cells. Clinically, it is characterized by nonhealing plaques that develop most commonly on the limbs. Porokeratosis, a premalignant condition, must be added to the list of potential cutaneous complications seen in immunosuppressed organ transplant recipients.

========================================================================
45.) Disseminated superficial porokeratosis and immunosuppression.
========================================================================
SO - Br J Dermatol 1988 Sep;119(3):375-80
AU - Neumann RA; Knobler RM; Metze D; Jurecka W
AD - Department of Dermatology II, University of Vienna, Austria.
PT - JOURNAL ARTICLE; REVIEW (18 references); REVIEW OF REPORTED CASES
AB - We present a patient who developed skin lesions typical of disseminated superficial porokeratosis (DSP) while on immunosuppressive therapy for pemphigus foliaceus. Phototesting with artificial light sources did not have any effect on the DSP lesions. The literature describing occurrence of DSP on immunosuppression is reviewed and possible pathogenetic mechanisms are discussed.

========================================================================
46.) Linear porokeratosis in two families with disseminated superficial actinic porokeratosis.
========================================================================
SO - Pediatr Dermatol 1987 Nov;4(3):209-14
AU - Commens CA; Shumack SP
AD - Skin and Cancer Foundation, Westmead, NSW, Australia.
PT - JOURNAL ARTICLE; REVIEW (23 references); REVIEW OF REPORTED CASES
AB - Two case reports of linear porokeratosis occurring in individuals or families with disseminated, superficial, actinic porokeratosis (DSAP) are presented. Linear porokeratosis and DSAP may be different expressions of one dominantly inherited condition. We reviewed the clinical features of linear porokeratosis and its association with other forms of porokeratosis. The linear form has a potential for carcinomatous change.

========================================================================
47.) Malignant disseminated porokeratosis.
========================================================================
SO - Arch Dermatol 1987 Nov;123(11):1521-6
AU - Brodkin RH; Rickert RR; Fuller FW; Saporito C
AD - Department of Medicine (Dermatology) University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark.
PT - JOURNAL ARTICLE
AB - A patient has been observed with a distinct form of disseminated porokeratosis. During the course of his disease, he developed changes in the porokeratosis lesions that included cellular atypia, dysplasia, and invasive squamous cell carcinoma. One of the squamous cell carcinomas produced regional and disseminated metastases, resulting in the death of the patient. Although malignancy has been previously described in the various types of porokeratosis, this is the first report of disseminated metastases and death in any form of this disease.

========================================================================
48.) Disseminated bilateral hyperkeratotic variant of porokeratosis Mibelli.
========================================================================
SO - Arch Dermatol Res 1987;279 Suppl:S38-47
AU - Marghescu S; Anton-Lamprecht I; Melz-Rothfuss B
AD - Hautklinik Linden, Federal Republic of Germany.
PT - JOURNAL ARTICLE
AB - Clinical, histopathological, and ultrastructural features of a new clinical variant of porokeratosis Mibelli (PM) are presented. We report a solitary case of a patient, male aged 62, who developed disseminated verrucous nodules on the buttocks and the lower extremities 3 years before diagnosis. Histopathologically, all specific signs of the keratinization disorder of PM were demonstrable; in addition, however, multiple cornoid lamellae were found at the margin as well as in the center of the lesions, which only in part showed relationships to the epidermal appendages. In the papillary dermis, numerous ectatic capillaries were conspicuous. Using electron microscopy the same specific abnormalities of the keratinization process as known from classical cases of PM could be demonstrated: autophagocytic cells that revealed perinuclear edematization and vacuolization, accumulation of autophagic vacuoles and heterolysosomes, and dyskeratotic corps ronds-like cells that become transformed to fibrillar or Civatte bodies. Problems of the classification, differential diagnosis, and pathomorphogenesis are discussed

========================================================================
49.) Porokeratosis plantaris, palmaris, et disseminata.
========================================================================
SO - Arch Dermatol 1986 Aug;122(8):890-1
AU - Marschalko M; Somlai B
PT - JOURNAL ARTICLE
AB - A 58-year-old man and his son presented with the clinical and histologic characteristics of porokeratosis plantaris, palmaris, et disseminata. Porokeratosis plantaris, palmaris, et disseminata also occurred in two other family members. Marked improvement was demonstrated with oral aromatic retinoid (Tigason) therapy. The differential diagnosis from the other types of porokeratosis is discussed. It is suggested that punctate porokeratosis is a form fruste of porokeratosis plantaris, palmaris, et disseminata.

========================================================================
50.) Porokeratosis of Mibelli: benzylhydrochlorothiazide-induced new lesions accompanied by eosinophilic spongiosis.
========================================================================
SO - J Am Acad Dermatol 1984 Aug;11(2 Pt 2):357-61
AU - Inamoto N; Watanabe T; Nakamura K
PT - JOURNAL ARTICLE
AB - We observed the course of development of porokeratosis stimulated by benzylhydrochlorothiazide (BHCTh) in normal-appearing skin of a patient with long-standing stable porokeratosis of Mibelli. A 72-year-old Japanese man had had porokeratosis of Mibelli for more than 50 years. During administration of BHCTh for 1 year because of his hypertension, a lichenoid drug eruption developed over the lesions of porokeratosis on the flexor aspects of his legs. Readministration of BHCTh by another physician for 6 months resulted in the occurrence of a similar drug eruption that converted into typical skin lesions of porokeratosis 8 weeks later. Serial microscopic examination suggested that BHCTh administration resulted in eosinophilic spongiosis and cornoid lamella formation, which developed into epidermal changes characteristic of porokeratos

========================================================================
51.) Congenital unilateral punctate porokeratosis.
========================================================================
SO - Am J Dermatopathol 1984 Feb;6(1):57-61
AU - Roberts LC; De Villez RL
PT - JOURNAL ARTICLE
AB - A 16-year-old girl with congenital unilateral punctate porokeratosis is described. The clinical and histopathologic findings are presented. The various clinical forms of porokeratosis and the differential diagnosis of this type of lesion are discussed. To our knowledge, this is the first case of congenital unilateral punctate porokeratosis to be reported.

========================================================================
52.) Punctate porokeratotic keratoderma--its occurrence with internal neoplasia.
========================================================================
SO - Clin Exp Dermatol 1994 Mar;19(2):139-41
AU - Bianchi L; Orlandi A; Iraci S; Spagnoli LG; Nini G
AD - Department of Dermatology, Tor Vergata University, Rome, Italy.
PT - JOURNAL ARTICLE
AB - Punctate porokeratotic keratoderma (PPK) represents a diffuse involvement of palms and soles by multiple, accuminate keratotic papules and plugs, histologically identified by parakeratotic cornoid lamellae. A possible association between PPK and internal malignancy has been previously noted by Herman in 1973. A patient with a 3-month history of PPK is described in which a bronchial carcinoma was recently diagnosed. This association led us to speculate that PPK could be a sign of internal neoplasia, as already established for other forms of palmoplantar keratoderma. We suggest that the presence of an underlying malignancy must be screened for when a diagnosis of PPK is proposed.

========================================================================
53.) Generalized eruptive porokeratosis of Mibelli with associated psoriasis.
========================================================================
SO - J Cutan Pathol 1975;2(4):203-13
AU - Eng AM; Kolton B
PT - JOURNAL ARTICLE
AB - A case of eruptive porokeratosis of Mibelli with diverse morphologic features, including circinate macular, circinate plaque and verrucous varieties is presented. No matter how variable the clinical presentation may be, the histologic hallmark of porokeratosis, the cornoid lamellae, is always present. The cornoid lamellae vary in height in relation to how prominent the thready ridge of the clinical lesion appears. Our patient also had psoriasis which initially masked the porokeratotic lesions both clinically and histologically. Awareness of the various clinical expressions of porokeratosis of Mibelli would 1) make unnecessary the segregation of certain forms of porokeratosis into separate entities, and 2) help in the recognition of less classical forms of porokeratosis.

========================================================================
54.) Cultured skin fibroblasts derived from three patients with disseminated superficial actinic porokeratosis (DSAP) are hypersensitive to the lethal effects of X-radiation but not to those of ultraviolet (UV) light.
========================================================================
SO - Exp Dermatol 1993 Aug;2(4):175-8
AU - Watanabe R; Otsuka F
AD - Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
PT - JOURNAL ARTICLE
AB - Disseminated superficial actinic porokeratosis (DSAP), skin lesions of which are known to be induced by ultraviolet (UV) light, does not develop into skin cancer as frequently as other types of porokeratosis (PK). To establish the cellular basis of this characteristic of DSAP, we examined the colony-forming ability of UVC light- or X-ray-irradiated cultured fibroblasts derived from DSAP patients' skin. Sensitivity to the lethal effects of UV light was not significantly different between 3 DSAP and 5 control cell strains. In contrast, DSAP cell strains were significantly hypersensitive to the lethal effects of X-radiation, although the sensitivity was less than that of cell strains from other types of PK patients. The results indicate that the actinic character of DSAP is not reflected in the cellular response to the lethal effects of UV light, but suggest that DSAP shares X-ray sensitivity, which is probably associated with the cancer-prone nature of PK.

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55.) Disseminated superficial porokeratosis in patients with pemphigus vulgaris ========================================================================
treated with steroids.
SO - Acta Derm Venereol Suppl (Stockh) 1979;59(85):59-61
AU - Feuerman EJ; Sandbank M
PT - JOURNAL ARTICLE
AB - In two patients with pemphigus vulgaris a rash identical with that seen in disseminated superficial porokeratosis developed on the trunk and extremities during periods in which they were receiving relatively high doses of steroids. Some time after reduction of the daily dose or discontinuance of treatment this rash disappeared. In one patient, however, this rash subsequently recurred twice, both times after reinstitution of treatment because of the reappearance of pemphigus lesions. It is suggested that disseminated superficial porokeratosis should be added to the list of possible skin eruptions developing as the result of the systemic use of corticosteroids at a high dose for a prolonged period of time, although the mechanism of its appearance in these cases is not yet clear.

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56.) Disseminated superficial porokeratosis developing after electron-beam total skin irradiation for mycosis fungoides.
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Clin Exp Dermatol 1996 Jul;21(4):310-2 (ISSN: 0307-6938)

Romani J; Pujol RM; Casanova JM; de Moragas JM [Find other articles with these Authors]

Hospital de la Santa Creu i Sant Pau, Spain.

A 74-year-old man with stage IB cutaneous T-cell lymphoma was treated with electron-beam total
skin irradiation in 1988. Seven years later, multiple disseminated lesions of porokeratosis
developed on the chest, extremities and abdomen. There was no family history of porokeratosis,
nor history of treatment with PUVA or of excessive sun exposure. Development of disseminated
porokeratosis on nonexposed sites suggests a direct role for the previous ionizing radiation.
Electron-beam total skin irradiation therapy should therefore be added to the list of possible
causative factors in the development of disseminated porokeratosis. 
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POROKERATOSIS, EL TRATAMIENTO /// POROKERATOSIS THE TREATMENT
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1.) Chemotherapy for disseminated actinic keratoses with 5-fluorouracil and isotretinoin.
2.) Porokeratosis of Mibelli: rapid recurrence of a large lesion after carbon dioxide laser treatment.
3.) Generalized linear porokeratosis treated with etretinate [letter]
4.) Porokeratosis plantaris, palmaris, et disseminata. Tigason Therapy
5.) Dexamethasone pulse treatment in disseminated porokeratosis of Mibelli [letter]
6.) Treatment of porokeratosis of Mibelli with CO2 laser vaporization versus surgical excision with split-thickness skin graft. A comparison.
7.) Disseminated superficial actinic porokeratosis responding to calcipotriol [letter]
8.) Reticulate porokeratosis--successful treatment with CO2-laser vaporization.
9.) Spiny keratoderma of the palms and soles.
10.) Porokeratosis of Mibelli with underlying hemangioma treated by the flashlamp-pumped pulsed dye laser.
11.) Linear porokeratosis: successful treatment with diamond fraise dermabrasion.
12.) Digitate keratoses--a complication of etretinate used in the treatment of disseminated superficial actinic porokeratosis.
13.) Exacerbation of porokeratosis during etretinate therapy.
14.) Etretinate in the treatment of disseminated porokeratosis of Mibelli.
15.) Carbon dioxide laser treatment of porokeratosis of Mibelli.
16.) Porokeratosis (Mibelli): treatment with topical 5-fluorouracil.
17.) [Ultrastructural study of Mibelli's porokeratosis. Pathogenic and therapeutic considerations in three cases]
18.) Cryosurgery of porokeratosis plantaris discreta.
19.) Disseminated superficial porokeratosis: Complete remission subsequent to discontinuation of immunosuppression
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1.) Chemotherapy for disseminated actinic keratoses with 5-fluorouracil and 
isotretinoin.
========================================================================
Sander CA; Pfeiffer C; Kligman AM; Plewig G
Department of Dermatology, Ludwig-Maximilians-Universitaet, Munich, Germany.
J Am Acad Dermatol (UNITED STATES) Feb 1997 36 (2 Pt 1) p236-8 ISSN: 0190-9622
Language: ENGLISH
Document Type: JOURNAL ARTICLE 
Journal Announcement: 9705
Subfile: INDEX MEDICUS
BACKGROUND: Disseminated actinic keratoses are a therapeutic problem. OBJECTIVE: 
Our purpose was to evaluate the efficacy of a combination of topical 5-fluorouracil 
twice a day and 20 mg of oral isotretinoin daily for disseminated actinic keratoses. 
METHODS: Twenty-seven patients who had disseminated actinic keratoses (3 women, 24 
men) were treated with 5-fluorouracil (5%) twice a day applied to the photodamaged 
area bearing actinic keratoses along with oral isotretinoin, 20 mg daily. The median 
treatment time was 21 days. RESULTS: Actinic keratoses disappeared and signs of 
photodamaged skin improved in all patients. Side effects were burning and itching as 
well as painful erosions during the final stage of treatment. CONCLUSION: The 
combination of topical 5-fluorouracil and isotretinoin is highly effective in the 
treatment of disseminated actinic keratoses on photodamaged skin.


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2.) Porokeratosis of Mibelli: rapid recurrence of a large lesion after carbon dioxide laser treatment.
========================================================================
SO - Pediatr Dermatol 1994 Sep;11(3):267-70
AU - McCullough TL; Lesher JL Jr
AD - Department of Dermatology, Medical College of Georgia, Augusta 30912-2900.
PT - JOURNAL ARTICLE
AB - Porokeratosis of Mibelli was diagnosed in a 9-year-old Afro-American girl. The lesion was rather large (4.1 x 2.0 cm) and had developed rapidly over the course of six months. Considering the potential for malignant transformation without neglecting the importance of cosmesis, carbon dioxide laser vaporization with curettage was chosen to ablate the lesion. Within two months the entire treated area revealed recurrent porokeratosis. Due to the aggressive nature of this lesion, complete excision was performed.

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3.) Generalized linear porokeratosis treated with etretinate [letter]
SO - Arch Dermatol 1995 Apr;131(4):496-7
AU - Goldman GD; Milstone LM
PT - LETTER
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4.) - Porokeratosis plantaris, palmaris, et disseminata. Tigason Therapy
========================================================================
SO - Arch Dermatol 1986 Aug;122(8):890-1
AU - Marschalko M; Somlai B
PT - JOURNAL ARTICLE
AB - A 58-year-old man and his son presented with the clinical and histologic characteristics of porokeratosis plantaris, palmaris, et disseminata. Porokeratosis plantaris, palmaris, et disseminata also occurred in two other family members. Marked improvement was demonstrated with oral aromatic retinoid (Tigason) therapy. The differential diagnosis from the other types of porokeratosis is discussed. It is suggested that punctate porokeratosis is a form fruste of porokeratosis plantaris, palmaris, et disseminata.

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5. )Dexamethasone pulse treatment in disseminated porokeratosis of Mibelli [letter]
SO - J Dermatol Sci 1994 Feb;7(1):71-2
AU - Verma KK; Singh OP
PT - LETTER
========================================================================

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6.) Treatment of porokeratosis of Mibelli with CO2 laser vaporization versus surgical excision with split-thickness skin graft. A comparison.
========================================================================
SO - J Dermatol Surg Oncol 1993 Mar;19(3):199-202
AU - Rabbin PE; Baldwin HE
AD - Department of Dermatology, State University of New York-Health Science Center, Brooklyn 11203.
PT - JOURNAL ARTICLE
AB - BACKGROUND. Porokeratosis of Mibelli has been treated with many topical surgical modalities in the past, including cold steel surgical excision and CO2 laser excision. We report a patient with an 8 x 10 cm2 plaque of porokeratosis on the dorsum of his hand, fingers, and web spaces. OBJECTIVE. To compare the treatment results of CO2 laser vaporization versus cold steel surgical excision with split-thickness skin graft placement. METHOD. The portions of the lesion overlying the metacarpophalangeal joints, fingers, and web spaces were treated with CO2 laser vaporization. The remainder of the lesion covering the dorsum of the hand was excised with cold steel, followed by placement of a split-thickness skin graft over that area. RESULTS. The portion of the porokeratosis lesion treated with CO2 vaporization healed with results cosmetically and functionally superior to the grafted area. CONCLUSION. Carbon dioxide laser vaporization, a more superficial treatment modality than CO2 laser excision, should be the preferred treatment modality for porokeratosis lesions.

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7.) Disseminated superficial actinic porokeratosis responding to calcipotriol [letter]
SO - Clin Exp Dermatol 1994 Jan;19(1):95
AU - Harrison PV; Stollery N
PT - LETTER
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8.) Reticulate porokeratosis--successful treatment with CO2-laser vaporization.
========================================================================
SO - Clin Exp Dermatol 1992 May;17(3):178-81
AU - Merkle T; Hohenleutner U; Braun-Falco O; Landthaler M
AD - Department of Dermatology, University of Munich, Germany.
PT - JOURNAL ARTICLE
AB - A 35-year-old male patient affected with extensive, itching skin lesions of reticulate porokeratosis is reported. He was successfully treated by CO2-laser vaporization.

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9.) Spiny keratoderma of the palms and soles.
========================================================================
SO - J Am Acad Dermatol 1992 May;26(5 Pt 2):879-81
AU - Osman Y; Daly TJ; Don PC
AD - Department of Dermatology, New York Medical College-Metropolitan Hospital Center, NY 10029.
PT - JOURNAL ARTICLE
AB - Six cases of a dermatosis characterized by "music box spine" keratotic papules limited to the palms and soles have been reported. The names that have been used for this type of dermatosis most commonly incorporate the term porokeratosis; for example, "porokeratosis palmaris et plantaris," or "punctuate porokeratotic keratoderma." We suggest the name "spiny keratoderma of the palms and soles," because the clinical, histologic, and electron microscopic features of this entity are distinct from porokeratosis. In addition, unlike porokeratosis, this entity has not shown malignant potential. We report a case of "spiny keratoderma of the palms and soles" and show that topical 5-fluorouracil is an effective treatment.

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10.) Porokeratosis of Mibelli with underlying hemangioma treated by the flashlamp-pumped pulsed dye laser.
========================================================================
SO - Cutis 1990 Nov;46(5):410-2
AU - Ashinoff R; Geronemus RG
AD - Department of Dermatology, New York University Medical Center, New York.
PT - JOURNAL ARTICLE
AB - Porokeratosis of Mibelli is a disorder of epidermal proliferation in which an abnormal clone of cells expands in a centrifugal manner. We present a case of porokeratosis of Mibelli with an underlying hemangioma that was treated with a 585 nm flashlamp-pumped pulsed dye laser. The underlying hemangioma responded well to laser therapy while the porokeratosis remained unchanged. The implications of this for the pathogenesis of porokeratosis and the specificity of the pulsed dye laser are discussed.

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11.) Linear porokeratosis: successful treatment with diamond fraise dermabrasion.
========================================================================
SO - J Am Acad Dermatol 1990 Nov;23(5 Pt 2):975-7
AU - Cohen PR; Held JL; Katz BE
AD - Department of Dermatology, College of Physicians and Surgeons Columbia University, New York, NY.
PT - JOURNAL ARTICLE
AB - A patient with linear porokeratosis was successfully treated with diamond fraise dermabrasion. Follow-up evaluation revealed an excellent cosmetic result with adequate repigmentation, no scarring, and no recurrence of lesions. Long-term follow-up will be necessary to determine whether dermabrasion treatment of linear porokeratosis provides adequate prophylaxis against the subsequent development of malignancy.

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12.) Digitate keratoses--a complication of etretinate used in the treatment of disseminated superficial actinic porokeratosis.
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SO - Clin Exp Dermatol 1990 Sep;15(5):370-1
AU - Carmichael AJ; Tan CY
AD - Skin Hospital, Edgbaston, Birmingham, UK.
PT - JOURNAL ARTICLE
AB - We report a patient with disseminated superficial actinic porokeratosis (DSAP) who developed digitate keratoses following treatment with etretinate. The aetiology of these digitate keratoses is discussed and their similarity to post irradiation conical keratoses (PICK) is highlighted.

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13.) Exacerbation of porokeratosis during etretinate therapy.
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SO - Acta Derm Venereol 1990;70(4):319-22
AU - Knobler RM; Neumann RA
AD - Department of Dermatology II, University of Vienna, Austria.
PT - JOURNAL ARTICLE
AB - Four patients with disseminated superficial porokeratosis (DSP) were treated with the oral aromatic retinoid etretinate. Using the standard dosage of 1 mg/kg/day, 3 patients showed exacerbation of cutaneous lesions 4-6 weeks after initiation of treatment. Because of additional severe generalized itching and discomfort in all patients, treatment had to be discontinued. The clinical exacerbation correlated with a significant increase in the lymphohistiocytic dermal infiltrate beneath the cornoid lamella. In our experience the use of etretinate triggered the exacerbation of porokeratotic lesions with associated side effects showing that their use is not necessarily of benefit as previously reported.

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14.) Etretinate in the treatment of disseminated porokeratosis of Mibelli.
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SO - Int J Dermatol 1985 May;24(4):258-60
AU - Hacham-Zadeh S; Holubar K
PT - JOURNAL ARTICLE
AB - A 30-year-old man with disseminated porokeratosis of Mibelli (DPKM) was treated with oral etretinate. The dose ranged between 75 mg/day and 50 mg/day for 21 weeks. An improvement of the lesions was observed, especially of very painful verrucous plaques of the left shin. No serious side effect was seen. The patient, who had been incapacitated, is now able to work. The benefit of long-term therapy of etretinate should be considered against its side effects.


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15.) Carbon dioxide laser treatment of porokeratosis of Mibelli.
========================================================================
SO - Lasers Surg Med 1985;5(6):603-6
AU - Groot DW; Johnston PA
PT - JOURNAL ARTICLE
AB - Porokeratosis of Mibelli, a disorder of keratinization, has in the past been difficult to treat, especially in its more extensive form. The carbon dioxide laser was used in the treatment of a patient with this disorder. The response was very favorable. Microscopic pathological investigation after treatment revealed no residual evidence of porokeratosis in the areas treated with the carbon dioxide laser. Thus, obliteration of this disease process using the carbon dioxide laser shows potential for future use.

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16.) Porokeratosis (Mibelli): treatment with topical 5-fluorouracil.
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SO - J Am Acad Dermatol 1983 Jan;8(1):107-10
AU - McDonald SG; Peterka ES
PT - JOURNAL ARTICLE
AB - The case history of a patient with a solitary classic porokeratosis (Mibelli) on the hand is described. The lesion cleared without scarring with the use of topical 5-fluorouracil until a strong dermatitis was elicited, thus avoiding surgery and attendant restriction of function. No sign of recurrence developed in the following 2 years. The potential for malignant change in porokeratosis (Mibelli) and its resistance to topical treatment as documented in the literature are also reviewed.

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17.) [Ultrastructural study of Mibelli's porokeratosis. Pathogenic and therapeutic considerations in three cases]
TT - [Etude ultrastructurale de la porokeratose de Mibelli. Considerations pathogeniques et therapeutiques a propos de trois cas.]
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SO - Ann Dermatol Venereol 1979 Sep;106(8-9):657-62
AU - Chavaz P; Carraux P; Laugier P
MC - English Abstract
PT - JOURNAL ARTICLE
AB - Report of three cases of porokeratosis Mibelli with emphasis on the ultrastructural changes of the entire epidermis underlying the cornoid lamella: autophagocytosis, filamentous degeneration, formation of "corps ronds". These phenomena are correlated with those of apoptosis (or intraepidermal cellular death with filamentous degeneration). Its role in the genesis of the cornoid lamella and in the degeneration of the lesions is discussed. In conclusion, on the basis of the reported observations and of the studies showing the action of retinoic acid and its derivatives on precancerous lesions, the authors propose to treat the actinic disseminated form of porokeratosis Mibelli with the aromatic derivative of retinoic acid (RO 10 9359).

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18.) Cryosurgery of porokeratosis plantaris discreta.
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SO - Arch Dermatol 1979 May;115(5):582-3
AU - Limmer BL
PT - JOURNAL ARTICLE
AB - Porokeratosis plantaris discreta is a distinct clinical and pathological entity often resistant to conservative management. Twenty-one lesions of porokeratosis plantaris discreta were treated cyrosurgically in 11 patients. Removal of the blister roof two weeks after cryosurgery with retreatment of any residual lesion proved an effective method for removal of such lesions without scarring. A cure rate of 90.5% was achieved using this technique.

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19.) Disseminated superficial porokeratosis: Complete remission subsequent to discontinuation of immunosuppression
AU: Tsambaos-D; Spiliopoulos-T
SO: J-Am-Acad-Dermatol. 28(4):651-2 1993
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DATA-MEDICOS/DERMAGIC-EXPRESS No (29) 13/01/99 DR. JOSE LAPENTA R. 
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