The Dermatomyositis / La Dermatomiositis

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****** DATA-MÉDICOS **********
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DERMATOMIOSITIS / DERMATOMYOSITIS
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****** DERMAGIC-EXPRESS No.52 *******
****** 13 MAYO DE 1.999 ***********
13 MAY 1.999
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EDITORIAL ESPAÑOL:
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Hola Amigos DERMAGICOS,,, el tema de hoy, LA DERMATOMIOSITIS, otra enfermedad del tejido conectivo cuyo tratamiento representa un verdadero reto, asociada a malignidad en algunos casos y de pronostico reservado en otros tantos. Pienso que estas 38 referencias son bastante ilustrativas.

Les recuerdo que DERMAGIC/ESPRESS esta disponible en CD-ROM, !!!

Desde Octubre 1998 hasta Mayo 1999, más de 1.000 referencias BibliográficaS, actualizadas repartidas en unos 50 temas,,,

PRÓXIMA EDICIÓN: ESCLEROSIS SISTÉMICA PROGRESIVA,

Dr. José Lapenta R.,,,

EDITORIAL ENGLISH:
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Hello DERMAGIC friends, today's topic, THE DERMATOMYOSITIS, another illness of the connective tissue whose treatment represents a true challenge, associated to malignancy in some cases, and in others of uncertain future.

I think that these 38 references are quite illustrative.

NEXT EDITIONS: SYSTEMIC SCLEROSIS (SCLERODERMA)

Greetings to ALL, !!

Dr. José Lapenta R.,,,

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DERMAGIC/EXPRESS(52)
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LA DERMATOMIOSITIS / THE DERMATOMYOSITIS
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1.) [Cyclophosphamide pulse therapy for refractory rheumatic diseases]
2.) Fulminant Pneumocystis carinii pneumonia in 4 patients with dermatomyositis.
3.) A retrospective study of 13 Oriental children with juvenile dermatomyositis.
4.) Pulsed intravenous methylprednisolone therapy for inflammatory myopathies:
evaluation of the effect by comparing two consecutive biopsies from the same muscle.
5.) [Generalized Mycobacterium avium-intracellulare infection due to immunosuppressive therapy of paraneoplastic dermatomyositis]
6.) Occurrence of polymyositis in the county of Gavleborg, Sweden.
7.) Cyclosporin a therapy in refractory juvenile dermatomyositis. Experience and longterm followup of 6 cases.
8.) Cyclosporine treatment for polymyositis/dermatomyositis: is it possible to rescue the deteriorating cases with interstitial pneumonitis?
9.) Progressive multifocal leukoencephalopathy during cyclosporine treatment. A case report.
10.) Update on therapy for refractory dermatomyositis and polymyositis.
11.) Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases.
12.) Lesions resembling malignant atrophic papulosis in a patient with dermatomyositis.
13.) Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis.
14.) Serum concentrations of carboxyterminal propeptide of type 1 procollogen and tissue inhibitor of metalloproteinase 1 in patients with dermatomyositis.
15.) Dermatomyositis: a dermatology-based case series.
16.) Microvascular changes in skeletal muscle in idiopathic inflammatorymyopathy.
17.) Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study.
18.) Ovarian malignancy in patients with dermatomyositis and polymyositis: a retrospective analysis of fourteen cases.
19.) Breast cancer and second primary ovarian cancer in dermatomyositis.
20.) [Vascular manifestations of dermatomyositis and polymyositis. Clinical, capillaroscopic and histological aspects]
21.) Long-term prognosis of 69 patients with dermatomyositis or polymyositis.
22.) Dermatomyositis and malignancy.
23.) Dermatomyositis. Disease associations and an evaluation of screening investigations for malignancy.
24.) [Dermatomyositis complicated by sarcoidosis]
25.) [Prognostic factors and predictive signs of malignancy in adult dermatomyositis]
26.) Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed.
27.) Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis?
28.) Association between bovine collagen dermal implants and a dermatomyositis or a polymyositis-like syndrome [see comments]
29.) MR imaging in amyopathic dermatomyositis.
30.) Case Report: amyopathic dermatomyositis associated with transformed malignant lymphoma.
31.) Malignancy in adult dermatomyositis.
32.) Treacher-Collins syndrome and co-existing dermatomyositis.
33.) [A clinical analysis of cutaneous type dermatomyositis]
34.) Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis.
35.) HCV and dermatomyositis: report of 5 cases of dermatomyositis in patients with HCV infection.
36.) [Dermatomyositis and cancer. A retrospective study]
37.) Amyopathic dermatomyositis (dermatomyositis sin´e myositis). Presentation of six new cases and review of the literature [see comments]
38.) Epstein-Barr virus strain type and latent membrane protein 1 gene deletions in lymphomas in patients with rheumatic diseases.
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1.) [Cyclophosphamide pulse therapy for refractory rheumatic diseases]
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Tateishi M; Taniguchi A; Moriguchi M; Hara M; Kashiwazaki S
Institute of Rheumatology, Tokyo women's Medical College.
Nihon Rinsho Meneki Gakkai Kaishi (JAPAN) Jun 1997 20 (3) p152-8
Language: JAPANESE Summary Language: ENGLISH
JOURNAL ARTICLE English Abstract
Journal Announcement: 9711

Subfile: INDEX MEDICUS

We studied the efficacy of cyclophosphamide pulse therapy (CYP) for refractory rheumatic diseases except for lupus nephritis. Thirty-five patients were included in all, that is 9 patients with systemic lupus erythematosus (SLE), 10 with rheumatoid arthritis (RA), 7 with polymyositis/dermatomyositis (PM/DM), 2 with progressive systemic sclerosis (PSS), 2 with anti-phospholipid antibody syndrome (APS), 1 with mixed connective tissue disease (MCTD), 1 with juvenile rheumatoid arthritis (JRA), 1 with Behcet's disease (BD), 1 with Wegener's granulomatosis (WG) and 1 with allergic granulomatous angiitis (AGA).

Moderate to marked improvement was noted in 4 patients with SLE (2 with CNS-lupus and 2 with vasculitis), 7 with RA (2 with interstitial pneumonia:IP, 2 with vasculitis and 2 with refractory arthritis), 4 with PM/DM (2 with IP and 2 with refractory myositis), 1 PSS with IP, 2 APS with thrombocytopenia, 1 JRA with vasculitis and BD with CNS disturbance. On the other hand, adverse reactions were observed in 9 out of 35 patients (25.8%). CYP should apply in the treatment for such refractory rheumatic diseases as CNS disturbance, vasculitis, IP and autoimmune thrombocytopenia.

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2.) Fulminant Pneumocystis carinii pneumonia in 4 patients with dermatomyositis.
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Bachelez H; Schremmer B; Cadranel J; Mouly F; Sarfati C; Agbalika F; Schlemmer B; Mayaud CM; Dubertret L
Institut de Recherche sur la Peau, Hopital Saint-Louis, Paris, France.
Arch Intern Med (UNITED STATES) Jul 14 1997 157 (13) p1501-3 ISSN: 0003-9926
Language: ENGLISH
Document Type: JOURNAL ARTICLE

Journal Announcement: 9710

Between 1989 and 1996, 4 cases of Pneumocystis carinii pneumonia (PCP) were observed in patients seronegative for the human immunodeficiency virus who were receiving corticosteroid therapy for dermatomyositis in our institution. These cases were considered unusual in light of the short delay of their onset after initiation of immunosuppressive therapy and their fulminant course: 3 of these patients died of PCP occurring during the first month of treatment with prednisone. In all 4 patients lymphopenia was observed before the initiation of corticosteroid treatment and low CD4 and CD8 cell counts were evident at the time of PCP.

These observations support the view of an increase in both the severity and incidence of PCP in patients without human immunodeficiency virus infection and question the need for a primary prophylaxis in patients with connective tissue diseases receiving high-dose corticosteroid therapy.

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3.) A retrospective study of 13 Oriental children with juvenile dermatomyositis.
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See Y; Giam YC; Chng HH
Department of Paediatrics, Alexandra Hospital, Singapore.
Ann Acad Med Singapore (SINGAPORE) Mar 1997 26 (2) p210-4 ISSN: 0304-4602
Language: ENGLISH
Document Type: JOURNAL ARTICLE

Journal Announcement: 9710

This is a retrospective study of 13 Oriental children with juvenile dermatomyositis (JDM). We reviewed data found in the hospital records of children diagnosed to have definite (n = 4), probable (n = 7) and possible (n = 2) JDM who presented over a 10- year period at 4 centres in Singapore and compared our results with the experience of others. We found an overall female preponderance (female to male ratio of 3.3:1) but an equal sex ratio in children below 5 years of age. The majority (92%) had insidious onset and good outcome.

Diagnosis was often delayed because of the insidious onset, and because weakness occurred late, was mild or absent. Only one child had an acute presentation and refractory course. She died despite aggressive therapy. Clinical features, complications and mainstay medication used were similar to Western studies.

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4.) Pulsed intravenous methylprednisolone therapy for inflammatory myopathies: evaluation of the effect by comparing two consecutive biopsies from the same muscle.
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Matsubara S; Hirai S; Sawa Y
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan. t- [email protected]
J Neuroimmunol (NETHERLANDS) Jun 1997 76 (1-2) p75-80 ISSN: 0165-5728
Language: ENGLISH

To examine the effect of pulsed intravenous methylprednisolone (IVMP) on polymositis (PM) and dermatomyositis (DM), two biopsies were taken from the same muscle before and after IVMP in one case each of PM and DM. After IVMP, numerous muscle fibers were seen undergoing regeneration.

Among all subsets of infiltrating cells that decreased in number, macrophages in the perivascular area decreased markedly. Also conspicuous was decrease of CD8+ cells in the endomysium in PM and that of B-cells in the perivascular area in DM.

Among infiltrating cells, frequency of those which expressed the signal transducers and activators of transcription 1 (STAT-1) fell considerably, but remained still abnormally high. Regions on the endothelial cells of blood vessels that expressed MHC antigens and intercellular adhesion molecules 1 (ICAM-1) also decreased but remained more widespread than normal. These results agreed with the favorable clinical effect of IVMP, but showed that many of the immunological parameters remained abnormal.

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5.) [Generalized Mycobacterium avium-intracellulare infection due to immunosuppressive therapy of paraneoplastic dermatomyositis]
Generalisierte Mycobacterium avium-intracellulare Infektion infolge immunsuppressiver Therapie einer paraneoplastischen Dermatomyositis.
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Schaller M; Korting HC; Meurer M; Schirren CG Dermatologische Klinik und Poliklinik, Ludwig-Maximilians-Universitat, Munchen.
Hautarzt (GERMANY) Feb 1997 48 (2) p118-21 ISSN: 0017-8470
Language: GERMAN Summary Language: ENGLISH
Document Type:
JOURNAL ARTICLE English Abstract

Subfile: INDEX MEDICUS

The ubiquitous Mycobacterium avium-intracellulare (MAI) is the most frequent cause of disseminated atypical mycobacteriosis in AIDS patients. MAI infections may develop in patients with other acquired immune defects, such as connective tissue disorders. In adults, the gastrointestinal and respiratory systems are most frequently affected. We report a patient with dermatomyositis receiving immunosuppressive therapy in whom only the skin and the skeletal system were affected by MAI. Because it presented with polymyositis-like symptoms, the infection was initially not identified and treated.

The MAI was cultured from a periarticular joint effusion from the right upper arm and from venous blood, as well as identified histologically in lesional skin. Resistance to antibiotics developed most likely because the patient failed to take oral antibiotics regularly. Because of an acute exacerbation of the tumor-associated dermatomyositis, immunosuppressive therapy was initiated, while the tuberculostatic therapy was continued. Using these therapies both diseases markedly improved. In patients with connective tissue disorders receiving longterm immunosuppressive therapy, especially when changes in symptoms and signs are observed, opportunistic infections such as MAI should be considered and included in the differential diagnosis.

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6.) Occurrence of polymyositis in the county of Gavleborg, Sweden.
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Weitoft T
Department of Internal Medicine, Lanssjukhuset Gavle, Sweden.
Scand J Rheumatol (NORWAY) 1997 26 (2) p104-6 ISSN: 0300-9742
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9707

Subfile: INDEX MEDICUS

Through register research 21 new cases (10 male, 11 female) of polymyositis/dermatomyositis during the period 1984-1993 in the county of Gavleborg, Sweden, were identified. The case records were studied retrospectively. Inclusion body myositis was found in three cases and overlap syndrome in seven cases. Three patients had associated malignant disease.

The annual incidence was estimated to 7.6 cases/million people. The clinical features are described. 19 patients were given steroid treatment, and azathioprin was the most used additive immunosuppressive drug. All patients could be followed for at least two years, and during this period three patients died (all of cancer disease). Remission and withdrawal of medication were achieved in five cases. The results of the study corresponds to previous investigations with similar design.

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7.) Cyclosporin a therapy in refractory juvenile dermatomyositis. Experience and longterm followup of 6 cases.
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Zeller V; Cohen P; Prieur AM; Guillevin L
Department of Internal Medicine, Hopital Avicenne, Universite Paris-Nord, Bobigny, France.
J Rheumatol (CANADA) Aug 1996 23 (8) p1424-7 ISSN: 0315-162X
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9707

Subfile: INDEX MEDICUS

OBJECTIVE: Cyclosporin A (CyA) has been reported to be an effective treatment of adult polymyositis (PM) and dermatomyositis (DM) refractory to corticosteroid therapy. We aimed to determine the effectiveness of CyA in the treatment of juvenile dermatomyositis (JDM) refractory to corticosteroids and cytotoxic agents. METHODS: Retrospective study of 6 patients with refractory JDM based on medical charts.

RESULTS: Better clinical control of the disease was obtained in all cases and corticosteroid doses could be markedly decreased or stopped. 4 patients relapsed, 3 after discontinuation of CyA, but recovered after its reinitiation. Trough CyA levels were maintained between 51 and 247 ng/ml, and side effects were rare and minor during a mean followup of 51.5 months.

CONCLUSION: CyA seems to be an effective treatment of JDM and should be considered in case of severe or refractory disease.

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8.) Cyclosporine treatment for polymyositis/dermatomyositis: is it possible to rescue the deteriorating cases with interstitial pneumonitis?
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Maeda K; Kimura R; Komuta K; Igarashi T
Second Department of Internal Medicine, Osaka Teishin Hospital, Japan.
Scand J Rheumatol (NORWAY) 1997 26 (1) p24-9 ISSN: 0300-9742
Language: ENGLISH

Document Type: JOURNAL ARTICLE

Polymyositis and dermatomyositis are inflammatory muscular diseases of unknown etiology which have interstitial pneumonitis as a serious complication. From 1987 to 1994, we used cyclosporine to treat 14 polymyositis/dermatomyositis patients (8 with and 6 without interstitial pneumonitis). In combination with prednisolone, cyclosporine was either added to or substituted for conventional cytotoxic drugs (azathioprine, cyclophosphamide and methotrexate). Cyclosporine was effective against both myositis and interstitial pneumonitis when used early in the course of the disease.

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9.) Progressive multifocal leukoencephalopathy during cyclosporine treatment. A case report.
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Gentile S; Sacerdote I; Roccatello D; Giordana MT
Divisione di Neurologia, Ospedale Giovanni Bosco, Torino, Italy.
Ital J Neurol Sci (ITALY) Oct 1996 17 (5) p363-6 ISSN: 0392-0461
Language: ENGLISH
Document Type: JOURNAL ARTICLE; REVIEW; REVIEW OF REPORTED CASES
Journal Announcement: 9705

Subfile: INDEX MEDICUS

A 45-year-old patient suffering from chronic renal insufficiency developed dermatopolymyositis. Since the patient did not respond to treatment with high doses of prednisone and immunoglobulin, concomitant cyclosporine A was added. Four months later, worsening signs of bilateral pyramidal disorder and an altered state of consciousness appeared. Serial computed tomography (CT) and magnetic resonance imaging (MRI) revealed multiple alterations of the cerebral white matter. Cyclosporine was then discontinued.

One month later, exitus occurred. Microscopic examination of the brain showed diffuse tumescence; histological examination revealed perivascular and diffuse lymphomonocyte infiltrations, areas of demyelination, astrocytes with bizarre nuclei, and oligodendrocytes with enlarged nuclei due to hyperchromatic inclusion. Morphological examination confirmed the presence of intranuclear icosahedral viral bodies. Progressive multifocal leukoencephalopathy was diagnosed. The literature contains only one report of an analogous case observed during a course of cyclosporine treatment for Wegener's granulomatosis. (20 References)

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10.) Update on therapy for refractory dermatomyositis and polymyositis.
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Villalba L; Adams EM
National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Arthritis and Rheumatism Branch, Bethesda, MD 20892-1820, USA.
Curr Opin Rheumatol (UNITED STATES) Nov 1996 8 (6) p544-51 ISSN: 1040-8711
Language: ENGLISH
Document Type: JOURNAL ARTICLE; REVIEW; REVIEW, TUTORIAL
Journal Announcement: 9705

Subfile: INDEX MEDICUS

Evaluation of new therapies for the inflammatory myopathies is complicated by the heterogeneity of these syndromes as well as by the lack of internationally accepted definitions of disease categories and assessments of disease activity and chronicity. This review covers our opinion of therapies and emphasizes the need for an early rehabilitation evaluation for these patients.

Oral corticosteroids are the first line of therapy for the inflammatory myopathies, but because of their side effects and the existence of a subset of patients in whom disease is controlled only with high-dose corticosteroids, we recommend considering the early use of a second-line immunomodulating agents or pulse intravenous methylprednisolone. A stepwise progression of therapies is suggested for patients who have increasing muscle weakness resulting from active disease. (61 References)

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11.) Partial lipodystrophy associated with juvenile dermatomyositis: report of two cases.
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Quecedo E; Febrer I; Serrano G; Martinez-Aparicio A; Aliaga A
Department of Dermatology, Hospital General Universitario, Valencia, Spain.
Pediatr Dermatol (UNITED STATES) Nov-Dec 1996 13 (6) p477-82 ISSN: 0736-8046
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9705
A 27-year-old woman and a 13-year-old girl diagnosed with juvenile dermatomyositis in childhood developed clinical findings of partial lipodystrophy 10 years after diagnosis. Exhaustive clinical and laboratory examinations showed an association with other abnormalities: hypertrichosis, steatohepatitis, and an abnormal insulin response to the glucose loading test in the first patient. Hypertrichosis, steatohepatitis, insulin-resistant diabetes mellitus, and acanthosis nigricans were observed in the second patient. Renal function was normal in both patients. Although a localized form of lipodystrophy has been reported associated with connective tissue disease (connective tissue lipoatrophy), the partial form has been infrequently described in association with juvenile dermatomyositis.

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12.) Lesions resembling malignant atrophic papulosis in a patient with dermatomyositis.
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Tsao H; Busam K; Barnhill RL; Haynes HA
Department of Dermatology, Harvard Medical School, Brookline, MA, USA.
J Am Acad Dermatol (UNITED STATES) Feb 1997 36 (2 Pt 2) p317-9 ISSN: 0190-9622
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9705

Subfile: INDEX MEDICUS

Many consider porcelain white atrophic papules as pathognomonic for malignant atrophic papulosis (MAP), or Degos' disease. During the past three decades, five patients with a collagen vascular disease have been reported to have MAP-like lesions as a manifestation of their underlying illness. We describe a patient with dermatomyositis who had porcelain-white atrophic papules resembling malignant atrophic papulosis.

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13.) Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis.
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Kasteler JS; Callen JP
Division of Dermatology, University of Louisville, KY, USA.
J Am Acad Dermatol (UNITED STATES) Jan 1997 36 (1) p67-71 ISSN: 0190-9622 Language: ENGLISH
Document Type: JOURNAL ARTICLE

Journal Announcement: 9704

BACKGROUND: The cutaneous manifestations of dermatomyositis can be the most prominent finding and are often difficult to treat.

OBJECTIVE: Our purpose was to determine whether low-dose methotrexate administered weekly in combination with other systemic therapies or as a sole systemic agent is effective in the treatment of the cutaneous disease in patients with dermatomyositis.

METHODS: We reviewed the records of 13 patients who received oral methotrexate in doses ranging from 2.5 to 30 mg weekly. Their skin lesions had not been completely responsive to sunscreens, topical corticosteroids, oral prednisone, oral antimalarial therapy, and, in one patient each, chlorambucil and azathioprine.

RESULTS: At the end of the study period, 4 of these 13 patients were free of all cutaneous manifestations of dermatomyositis, and another four had almost complete clearing. In the remaining five patients, methotrexate induced moderate clearing of their cutaneous lesions. In all patients, the addition of methotrexate allowed reduction or discontinuation of other therapies such as prednisone. All patients tolerated the methotrexate with minimal toxicity.

CONCLUSIONS: Low-dose oral methotrexate administered weekly is effective in treatment of the cutaneous manifestations of dermatomyositis and frequently enables a reduction or discontinuation of corticosteroid therapy.

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14.) Serum concentrations of carboxyterminal propeptide of type 1 procollogen and tissue inhibitor of metalloproteinase 1 in patients with dermatomyositis.
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Author
Kubo M; Kikuchi K; Fujimoto M; Tamaki K
Address
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan. [email protected]
Source
Arch Dermatol Res, 290(5):253-7 1998 May

Abstract

Serum levels of carboxyterminal propeptide of type I procollagen (PICP) and aminoterminal propeptide of type I procollagen (PINP) have been used as indices of collagen synthesis in patients with various fibrotic diseases during the active stages.

One of the suggested contributory factors to the development of tissue fibrosis is a decrease in collagenase activity, which may be related to levels of serum tissue inhibitors of metalloproteinases-1 (TIMP-1). In this study, the serum levels of PICP and PINP in 20 patients with dermatomyositis and 29 control subjects and of TIMP-1 in 29 patients with dermatomyositis and 29 control subjects were measured using an enzyme-linked immunosorbent assay (ELISA) or a radioimmunoassay (RIA). We found that the mean PICP level in patients with dermatomyositis was significantly higher than that in normal controls (mean +/- SD 326+/-76 ng/ml vs 135+/-88 ng/ml; P < 0.001). In 60% of dermatomyositis patients, the serum PICP level was elevated (more than 311 ng/ml, i.e. 2 x SD above the mean control value). Elevated serum PICP levels were correlated with the incidence of elevated serum creatine kinase levels in patients with dermatomyositis.

The serum concentration of PINP was not elevated in comparison with that of the normal control subjects (mean +/- SD 36+/-30 ng/ml vs 63+/-34 ng/ ml). The mean TIMP-1 level in the patients with dermatomyositis was also significantly higher than in the normal control subjects (mean +/- SD 438+/-328 ng/ml vs 163+/-63 ng/ml; P < 0.001). In 59% of dermatomyositis patients, the mean serum TIMP-1 level was elevated (more than 289 ng/ml, i.e. 2 x SD above the mean control value). Serum PICP and TIMP-1 levels might be useful for detecting disease activity or severity in dermatomyositis.

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15.) Dermatomyositis: a dermatology-based case series.
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Author
Dawkins MA; Jorizzo JL; Walker FO; Albertson D; Sinal SH; Hinds A
Address
Department of Dermatology, Bowman Gray School of Medicine of Wake Forest University, Winston-Salem, North Carolina 27157-1071, USA.
Source
J Am Acad Dermatol, 38(3):397-404 1998 Mar

Abstract

BACKGROUND: Dermatomyositis is associated with significant morbidity and occasional mortality. Currently there is no consensus on treatment for patients with dermatomyositis.

OBJECTIVE: Our purpose was to review the clinical features and response to therapy of patients with dermatomyositis and compare these data with previous series of patients with dermatomyositis/polymyositis.

METHODS: Clinical characteristics of 65 patients seen during a 10-year period were reviewed retrospectively. Twenty-one of these patients were enrolled in a prospective, uncontrolled study of treatment with high-dose prednisone followed by slow tapering.

RESULTS: Clinical features were similar to those previously described; however, muscle strength at diagnosis was on average greater in patients in this series than in patients previously reported. Malignancy was present in 5 of 43 adult patients (12%), but was not found in patients with juvenile dermatomyositis. Another connective tissue disease was present in 19% of patients. Twelve patients had dermatomyositis sine myositis. Eighteen of 21 patients (85%) in the prednisone study group had resolution of myositis.

CONCLUSION: Patients with dermatomyositis in this series had less active myositis at presentation, but were otherwise similar to patients with dermatomyositis/polymyositis previously reported. Treatment with high-dose daily prednisone followed by slow tapering was effective.

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16.) Microvascular changes in skeletal muscle in idiopathic inflammatory myopathy.
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Author
Estruch R; Grau JM; Fern´andez-Sol´a J; Casademont J; Monforte R; Urbano-M´arquez A
Address
Department of Internal Medicine, Hospital Cl´inic, University of Barcelona, Spain.
Source
Hum Pathol, 23(8):888-95 1992 Aug

Abstract

Open deltoid muscle biopsy specimens from patients with idiopathic adult dermatomyositis, paraneoplastic dermatomyositis, childhood dermatomyositis, and idiopathic polymyositis, and from control patients were studied. Qualitative and morphometric capillary analysis by phase and electron microscopy was carried out. In the morphologic analysis the most striking difference was the presence of capillary damage and a higher capillary depletion in dermatomyositis as well as a higher capillary density in polymyositis.

By electron microscopy, capillaries from patients with dermatomyositis showed mainly microtubuloreticular structures, loss of endothelial plasma membranes, and the appearance of abnormal cytoplasmic organelles. In contrast, capillaries from patients with polymyositis exhibited only minimal changes. By morphometric analysis, muscle capillaries in dermatomyositis had a significantly higher mean endothelial thickness than those in polymyositis.

Finally, a significant topographic association between capillary damage and muscle fiber changes was observed only in patients with dermatomyositis. On the other hand, paraneoplastic dermatomyositis showed fewer structural and morphometric capillary changes than the other forms of dermatomyositis. We conclude that dermatomyositis is characterized by microvascular alterations that are absent in polymyositis. The topographic proximity of capillary changes to muscle fiber injury suggests that capillary damage may play a role in the pathogenesis of the muscle lesions observed in patients with dermatomyositis.

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17.) Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study.
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Author
Sigurgeirsson B; Lindel¨of B; Edhag O; Allander E
Address
Department of Dermatology, Karolinska Hospital, Stockholm, Sweden.
Source
N Engl J Med, 326(6):363-7 1992 Feb 6

Abstract

BACKGROUND. An association between polymyositis and cancer was first proposed in 1916, but the existence of the association has been disputed. An association between dermatomyositis and cancer is better accepted, but its magnitude is not known.

METHODS. We undertook a study to provide accurate estimates of the risk of cancer in patients with dermatomyositis or polymyositis. We studied the incidence of cancer and the rate of mortality from cancer in a population-based cohort of 788 patients with dermatomyositis or polymyositis in Sweden from 1963 through 1983. The results were compared with those for the general population.

RESULTS. Among the 396 patients with polymyositis, 42 cancers were diagnosed at the same time or after polymyositis was diagnosed in 37 patients (9 percent). The relative risk of cancer was 1.8 (95 percent confidence interval, 1.1 to 2.7) in the male patients and 1.7 (95 percent confidence interval, 1.0 to 2.5) in the female patients. Eighty-four males and 85 females died, and in 24 of these cases (14 percent) cancer was the principal cause of death.

The mortality ratio (the rate of mortality from cancer in these patients as compared with that in the general population) was 0.90 (95 percent confidence interval, 0.6 to 1.4). Among the 392 patients with dermatomyositis, 61 cancers were diagnosed at the same time or after dermatomyositis was diagnosed in 59 patients (15 percent). The relative risk of cancer was 2.4 (95 percent confidence interval, 1.6 to 3.6) in the male patients and 3.4 (95 percent confidence interval, 2.4 to 4.7) in the female patients. Fifty-seven males and 110 females died, and in 67 of these cases (40 percent) cancer was the principal cause of death (mortality ratio, 3.8; 95 percent confidence interval, 2.9 to 4.8).

CONCLUSIONS. The risk of cancer is increased in patients with polymyositis or dermatomyositis. In patients with dermatomyositis there is also a higher rate of mortality from cancer.

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18.) Ovarian malignancy in patients with dermatomyositis and polymyositis: a retrospective analysis of fourteen cases.
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Author
Davis MD; Ahmed I
Address
Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
Source
J Am Acad Dermatol, 37(5 Pt 1):730-3 1997 Nov

Abstract

BACKGROUND: Dermatomyositis and polymyositis can be associated with an underlying malignancy.

OBJECTIVE: Our purpose was to describe a group of patients with dermatomyositis and polymyositis in whom ovarian carcinoma was diagnosed and to evaluate further the characteristics of the association between these diseases.

METHODS: A cross-sectional retrospective review identified 14 patients in a 45-year period (1950 to 1995) with dermatomyositis and polymyositis in whom an underlying ovarian malignancy was diagnosed. Clinical and laboratory data of these patients were reviewed, and immunofluorescent and hematoxylin-eosin-stained skin sections were examined.

RESULTS: The mean age at diagnosis of dermatomyositis and polymyositis was 59 years. Of the 14 patients, 11 had dermatomyositis, two had polymyositis, and one had an overlap disease. The 12 patients with dermatomyositis had distinctive cutaneous lesions. In eight, cutaneous biopsy specimens demonstrated variable histologic changes. Thirteen patients had signs of proximal muscle weakness, and myopathy was further demonstrated by electromyographic testing in 10. Creatine kinase levels were increased in 6 of the 10 patients tested. Dysphagia was present in five patients, but esophageal dysmotility was not demonstrated in three. Dermatomyositis and polymyositis preceded the ovarian malignancy in nine patients, were concomitant with it in four, and succeeded the ovarian disease in one.

CONCLUSION: Physical examination and imaging techniques failed to detect early ovarian cancer in our patients with dermatomyositis and polymyositis. When detected (usually by abdominopelvic examination, in two by computed tomographic examination), it was advanced, and survival was poor.

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19.) Breast cancer and second primary ovarian cancer in dermatomyositis.
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Author
Voravud N; Dimopoulos M; Hortobagyi G; Ross M; Theriault R
Address
Department of Medical Oncology, University of Texas M. D. Anderson Cancer Center, Houston 77030.
Source
Gynecol Oncol, 43(3):286-90 1991 Dec

Abstract

We report six female patients with breast cancer who developed dermatomyositis and compare our data with those from other reports. The development of dermatomyositis in two patients led to the discovery of a second primary ovarian carcinoma, whereas the development of dermatomyositis in another two patients led to the discovery of recurrent breast cancer. In three patients the diagnosis of dermatomyositis preceded the diagnosis of breast cancer, while the rest developed dermatomyositis after the diagnosis of breast cancer.

A parallel clinical course of dermatomyositis and breast cancer was seen in only one patient. Coexisting dermatomyositis and breast cancer is a rare phenomenon, and dermatomyositis that develops during the course of breast cancer may indicate the occurrence of a second primary malignancy or recurrent breast cancer. The onset of dermatomyositis may precede, coincide with, or follow the diagnosis of breast cancer. The clinical course of dermatomyositis sometimes, but not always, parallels the course of breast cancer. There are no specific clinical or laboratory markers to distinguish patients with dermatomyositis who have malignancy from those without cancer.

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20.) [Vascular manifestations of dermatomyositis and polymyositis. Clinical, capillaroscopic and histological aspects]
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Author
Leteurtre E; Hachulla E; Janin A; Hatron PY; Brouillard M; Devulder B
Address
Service de m´edecine interne, CHRU, h^opital Claude-Huriez, Lille.
Source
Rev Med Interne, 15(12):800-7 1994

Abstract

Polymyositis is characterized by a T-cell-mediated and MHC-I-restricted cytotoxic process, whereas dermatomyositis is a primitively vascular disease with microangiopathy mediated by the complement C5b-9 membranolytic attack complex.

We have tried to estimate the frequency of vascular abnormalities in polymyositis as defined by Bohan and Peter. We have retrospectively studied 17 patients with dermatomyositis and 15 patients with polymyositis. Vascular abnormalities have been defined by clinical, capillaroscopic and histologic (muscle biopsy and minor salivary glands biopsy) features.

After clinical features, 5/17 dermatomyositis had a Raynaud's phenomenon, against 6/15 polymyositis. Digital necrosis has been observed for 2/17 dermatomyositis and 2/15 polymyositis. In capillaroscopy, 14/17 dermatomyositis had a microangiopathy with or no enlarged capillary loops, against 7/15 polymyositis. None of these polymyositis had enlarged capillary loops.

The muscle biopsy showed a predominantly perivascular or perimysial inflammatory infiltrate (vascular process) for 10/16 dermatomyositis against 4/13 polymyositis; a perifascicular atrophy for 3/16 dermatomyositis against 2/13 polymyositis. The histological study of minor salivary glands, showed vascular lesions for 2/11 dermatomyositis and for 1/8 polymyositis.

Finally, Bohan and Peter's classification is now inadequate to distinguish between dermatomyositis and polymyositis. Indeed, some dermatomyositis sine dermatitis, may exist and be recognized by their vascular features. To distinguish between dermatomyositis and polymyositis is important, to evaluate the risk of cancer which is more frequent in dermatomyositis.

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21.) Long-term prognosis of 69 patients with dermatomyositis or polymyositis.
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Author
Maugars YM; Berthelot JM; Abbas AA; Mussini JM; Nguyen JM; Prost AM
Address
Department of Rheumatology, Nantes University Hospital Center, France.
Source
Clin Exp Rheumatol, 14(3):263-74 1996 May-Jun

Abstract

OBJECTIVES: To assess the long-term prognosis of dermatomyositis and pol myositis.

METHODS: 69 patients with dermatomyositis or polymyositis were selected according to the diagnostic criteria of Bohan and Peter and were followed up for a minimum of 6.3 years (for surviving patients) (mean 11.6 years). Clinical and biological features, and pulmonary and muscle parameters were considered as prognostic factors for death. Functional disability was assessed using a 4-stage grading system.

RESULTS: 30 deaths (43.5%) occurred mainly due to cardiovascular (8), pulmonary (8), carcinomatous (5) and iatrogenic complications (5). Survival rates were 82.6% at 1 year, 73.9% at 2.66, 7% at 5 and 55.4% at 9. Significant prognostic factors for death (Cox model with time-dependent covariates) were old age (p < 0.0001), dysphonia (p < 0.001), pulmonary interstitial fibrosis (p < 0.02), absence of dysphagia (p < 0.02) and asthenia-anorexia (p < 0.05).

Dermatomyositis and polymyositis subgroups had slightly different significant prognostic factors for death: old age, cancer, pulmonary interstitial fibrosis and asthenia-anorexia for dermatomyositis; old age, failure to improve muscle strength in response to treatment after one month, and the absence of myalgia as presenting symptom for polymyositis. At the end of the follow-up, 33/39 surviving patients (84.6%) had no or insignificant muscular disability, whereas 3 children were bedridden due to generalized calcinosis.

CONCLUSIONS: High mortality occurred in the first year, and the survival rate decreased continually up to 9 years. The main prognostic factor for death is old age, but dermatomyositis and polymyositis must be considered separately. General features (pulmonary fibrosis, cancer, asthenia-anorexia) are involved in dermatomyositis, whereas muscular symptoms are the most significant in polymyositis. The long-term functional prognosis was fairly good, except for generalized calcinosis, which tended to occur in childhood dermatomyositis.

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22.) Dermatomyositis and malignancy.
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Author
Bernard P; Bonnetblanc JM
Address
Department of Dermatology, Dupuytren University Hospital, Limoges, France.
Source
J Invest Dermatol, 100(1):128S-132S 1993 Jan

Abstract

During the past 12 years, many studies applying strict diagnostic criteria have been published that have attempted to settle the controversy about the reality of the association between dermatomyositis and malignancy. Although retrospective, recent studies have shown an increased incidence of malignancy among patients with dermatomyositis when compared with controls without myositis. In contrast, an increased frequency of malignancy in dermatomyositis as compared to polymyositis still has to be demonstrated. In most cases, malignant disease precedes or occurs concurrently with dermatomyositis and is discovered on the basis of clinical signs, symptoms, and routine screening laboratory tests.

The types of neoplasms found in association with dermatomyositis parallel those observed in the general population. A possible link between dermatomyositis and an underlying malignancy remains largely hypothetical at a biologic level, although cellular immunity abnormalities may provide a direction for future investigations. Prospective epidemiologic studies using the case-control methods and cohort analysis remain necessary 1) to rigorously demonstrate the reality and to study the nature of the association between dermatomyositis and malignancy, and 2) to clarify the optimal screening strategies for malignant neoplasms in patients with dermatomyositis.

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23.) Dermatomyositis. Disease associations and an evaluation of screening investigations for malignancy.
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Author
Cox NH; Lawrence CM; Langtry JA; Ive FA
Address
Department of Dermatology, Royal Victoria Infirmary, Newcastle-upon-Tyne, England.
Source
Arch Dermatol, 126(1):61-5 1990 Jan

Abstract

Fifty-three adult patients (19 men, 34 women) with dermatomyositis were studied. Two had dermatomyositis associated with benign disorders. Twenty-three (43%) had a malignancy; the risk of malignancy increased with age, but there was no sex difference.

Seven malignancies were recurrences and 9 were diagnosed during investigation of dermatomyositis; these 16 were suspected clinically or from abnormal results of simple investigations. Extensive screening tests did not increase the number of malignancies diagnosed. In 7 patients, a diagnosis of malignancy was made more than 9 months after onset of dermatomyositis, although a relationship between malignancy and dermatomyositis was uncertain in two cases; the diagnosis of gynecological malignancy was missed in 2 patients despite appropriate investigations, 1 patient had poorly controlled dermatomyositis, and in 2 patients late diagnosis of malignancy was due to failure to reinvestigate relapse of previously stable dermatomyositis.

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24.) [Dermatomyositis complicated by sarcoidosis]
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Author
Takano Y; Oida K; Kohri Y; Taguchi Y; Tomii K; Matsumura Y; Mino M; Gohma I; Kobashi Y; Yuba Y
Address
Department of Respiratory Medicine and Pathology, Tenri Hospital.
Source
Nihon Kyobu Shikkan Gakkai Zasshi, 34(11):1255-9 1996 Nov

Abstract

We encountered a patient with dermatomyositis complicated by sarcoidosis. A 57-year-old woman was admitted to our hospital because of fever dry cough, and myalgias. There were reticular shadows on her chest X-ray film. Although the typical skin rash and myositis suggested the diagnosis of dermatomyositis biopsy specimens from a salivary gland, muscle, and lung revealed noncaseating granulomas as well. Uveitis was also noted. These findings suggested the coexistence of sarcoidosis with dermatomyositis.

Examination of the lung-biopsy specimens showed interstitial pneumonia compatible with dermatomyositis, except for the granuloma. The typical rash of dermatomyositis and pathological findings of the lung specimen were inconsistent with sarcoidosis. Therefore we concluded that this patient had both dermatomyositis and sarcoidosis. This case sheds new light on the importance of pathological examinations.

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25.) [Prognostic factors and predictive signs of malignancy in adult dermatomyositis]
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Author
Gallais V; Crickx B; Belaich S
Address
Service de Dermatologie, H^opital Bichat, Paris.
Source
Ann Dermatol Venereol, 123(11):722-6 1996

Abstract

INTRODUCTION: Prognosis in dermatomyositis is severe, partly due to the development of cancer. The aim of this study was to identify factors predicting cancer development and assess factors predicting reduced survival rate.

PATIENTS AND METHODS: A retrospective analysis of 32 cases of dermatomyositis diagnosed on the basis of the Bohan and Peter criteria was performed. Diagnosis was certain in 7 cases, probable in 13 and possible in 5. There were thus 7 cases of pure cutaneous dermatomyositis. Clinical and laboratory data were compared between patients with and without cancer and between deceased and surviving patients.

RESULTS: Overall mortality was 37.5% at 4 years, confirming the gravity of dermatomyositis. Malignancy developed in 9 patients (28.1%) leading to death in all cases, within 18 months in 8. Amyopathic dermatomyositis was observed in 2 of these patients. Necrotic skin ulcerations (p < 0.01) and pruritis (p < 0.05) were significant predictive factors for the development of cancer. Poor prognosis factors were malignancy (p < 0.001), necrotic skin ulcerations (p < 0.01), and pruritis (p < 0.05).

CONCLUSION: Prognosis is poor in certain sub-groups of patients with dermatomyositis. Such patients can be identified on the basis of skin lesions, notable necrotic ulcerations.

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26.) Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed.
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Author
Kasteler JS; Callen JP
Address
Division of Dermatology, University of Louisville, School of Medicine, KY.
Source
JAMA, 272(24):1939-41 1994 Dec 28

Abstract

OBJECTIVE--To characterize scalp involvement in patients with dermatomyositis.

DESIGN--Case series.

PATIENTS--All patients with dermatomyositis seen in our office between 1988 and mid 1993. Patient inclusion in this study required fulfillment of three or more of Bohan and Peter's criteria for dermatomyositis.

RESULTS--Of 17 patients with the diagnosis of dermatomyositis, scalp involvement was present in 14. Five of the 14 patients with scalp involvement were diagnosed as having scalp psoriasis or seborrheic dermatitis before progression of their disease or tissue examination revealed the diagnosis of dermatomyositis. In all patients, the scalp involvement was manifested as atrophic, erythematous, scaly plaques. In addition, alopecia was noted in six of the 14 patients. Treatment of cutaneous involvement included sun avoidance, topical corticosteroids and/or antimalarials, and/or methotrexate.

CONCLUSIONS--Recognition of this process is important because scalp involvement is often overlooked, may be misdiagnosed initially, and can be the presenting complaint in some patients with dermatomyositis.

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27.) Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis?
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Author
Stonecipher MR; Jorizzo JL; White WL; Walker FO; Prichard E
Address
Department of Dermatology, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC 27157.
Source
J Am Acad Dermatol, 28(6):951-6 1993 Jun

Abstract

BACKGROUND: Dermatomyositis sine myositis may have various connotations. Controversy exists as to nomenclature, degree of evaluation required, therapy, and course (e.g., does true dermatomyositis of the skin only exist?).

OBJECTIVE: The purpose of this study was to assess prospectively patients with the clinicopathologic features of dermatomyositis and normal muscle enzyme serum levels to determine their course in terms of the onset of muscle disease.

METHODS: Thirteen patients were studied by complete history and clinical examination, laboratory studies, electromyography, and skin and muscle biopsy. They were observed for 1 to 6 years.

RESULTS: Patients were classifiable into three groups: (1) cutaneous changes only, (2) cutaneous changes only at baseline with subsequent development of myositis, and (3) cutaneous changes with normal muscle enzyme serum levels at baseline but with myositis demonstrated by electromyography and/or muscle biopsy specimens.

CONCLUSION: Significantly different prognostic and therapeutic implications are present in patients with dermatomyositis with normal muscle enzyme serum levels depending on the results of electromyography, muscle biopsy, and clinical observation.

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28.) Association between bovine collagen dermal implants and a dermatomyositis or a polymyositis-like syndrome [see comments]
=====================================================================
Author
Cukier J; Beauchamp RA; Spindler JS; Spindler S; Lorenzo C; Trentham DE
Address
Baylor College of Medicine, Department of Plastic Surgery, Houston, TX 77030.
Source
Ann Intern Med, 118(12):920-8 1993 Jun 15

Abstract

OBJECTIVE: To determine whether an excess incidence of dermatomyositis or polymyositis or both exist in patients treated with injectable bovine collagen implants and to characterize the clinical picture. DESIGN: Historical cohort study (July 1980 through June 1988).

PATIENTS: Patients were identified from personal experience or adverse reaction reports received by the manufacturer.

SETTING: An 8-year period in the United States during which approximately 345,000 patients received implants.

RESULTS: Eight patients with dermatomyositis and an additional patient with polymyositis were identified from approximately 345,000 patients receiving injectable bovine collagen implants from July 1980 through June 1988. The nine patients with dermatomyositis or polymyositis were diagnosed an average of 6.4 months (range, 0.7 to 24.9 months) after collagen implant or skin test exposure or both. Eight of the nine patients had a delayed-type hypersensitivity response at the test or treatment sites or both, and five of six patients tested were found to have increased serum antibodies to collagen.

Compared with the general population, the incidence of dermatomyositis or polymyositis among collagen-treated patients was statistically increased (standardized incidence ratio, 5.05; 95% CI, 2.31 to 9.59; P < 0.0001). A similar analysis of the eight dermatomyositis case patients produced a standardized incidence ratio of 18.8 (CI, 8.1 to 37.0; P < 0.0001). Using a Monte Carlo simulation, an interval of 6.4 months or less from exposure to onset of disease was found to be an extremely rare event, occurring less than 72 times per one million simulation trials (CI, 57 to 91).

CONCLUSIONS: Because these data suggest that an immunologic response to bovine type I or type III collagen or both caused this dermatomyositis or polymyositis-like syndrome, the risks versus benefits for the cosmetic use of collagen implants should be reassessed.

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29.) MR imaging in amyopathic dermatomyositis.
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Author
Lam WW; Chan H; Chan YL; Fung JW; So NM; Metreweli C
Address
Department of Diagnostic Radiology and Organ Imaging, Chinese University of Hong Kong, Shatin, Hong Kong.
Source
Acta Radiol, 40(1):69-72 1999 Jan

Abstract

PURPOSE: Amyopathic dermatomyositis is a distinct clinical entity with cutaneous involvement but no myopathy. We conducted a prospective study to investigate the role of MR imaging in these patients.

MATERIAL AND METHODS: Out of 40 Chinese patients presenting with dermatomyositis, based on clinical assessment and normal serum muscle enzymes 10 were diagnosed as having amyopathic dermatomyositis. These 10 patients underwent MR imaging for evaluation of any subclinical muscle involvement.

RESULTS: Three patients demonstrated abnormal signal intensity in muscles on both T2- and fat suppression sequences. Thus, one-third of patients with dermatomyositis and clinically normal muscles may have detectable muscle inflammation on MR images, indicating that MR has a potential role for locating the relevant biopsy site and for longitudinal follow up. Six of the 10 patients had malignant disease diagnosed before or after diagnosis of the cutaneous manifestation. Nasopharyngeal carcinoma was the most common malignant disease in this group of patients.

CONCLUSION: MR imaging is recommended for demonstrating subclinical muscle involvement in patients with the clinical diagnosis of amyopathic dermatomyositis. We also recommend screening for malignancy, particularly nasopharyngeal carcinoma, in Southern Chinese patients with dermatomyositis.

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30.) Case Report: amyopathic dermatomyositis associated with transformed malignant lymphoma.
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Author
Osman Y; Narita M; Kishi K; Fujiwara H; Fukuda T; Koike T; Shibata A
Address
First Department of Internal Medicine, Niigata University School of Medicine, Japan.
Source
Am J Med Sci, 311(5):240-2 1996 May

Abstract

Amyopathic dermatomyositis is a disease of unknown origin characterized by the specific skin lesions of dermatomyositis but without clinical or laboratory evidence of myopathy. During the past 15 years, a great controversy between the different reports concerning a possible association of dermatomyositis with malignancy has been noted. In this report, the authors describe a patient with amyopathic dermatomyositis who presented first with a benign hyperplasia of the lymph node, which finally transformed into frank malignant lymphoma. In addition to follow-up care, screening tests to search for occult malignancy in patients with amyopathic dermatomyositis (or dermatomyositis) are recommended.

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31.) Malignancy in adult dermatomyositis.
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Author
Leow YH; Goh CL
Address
National Skin Centre, Singapore.
Source
Int J Dermatol, 36(12):904-7 1997 Dec

Abstract

BACKGROUND: Dermatomyositis has been reported to be associated with malignancies in 15%-34% of patients in Western countries, but in as many as two-thirds of patients in Singapore. The aim of this study was to determine whether a diagnostic measure could be helpful in the diagnosis of a malignancy in patients with dermatomyositis.

METHODS: This is a retrospective study on 38 adult patients with dermatomyositis that were seen over a 6-year period from 1989 to 1994.

RESULTS: All the patients presented with cutaneous features that suggest the clinical diagnosis of dermatomyositis; however, not all cases show all the key features of the disease. Of the studied patients, 86.8% were noted to have photosensitivity as a key cutaneous presentation. Thirty (78.9%) of our patients were above the age of 40 years, and 12 (31.6%) of these were found to have an associated malignancy. Nasopharyngeal carcinoma was the most commonly associated malignancy (38.4%) in our study population.

CONCLUSIONS: In our study population, otorhinolaryngologic screening is an essential investigation for the evaluation of dermatomyositis in association with malignancy.

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32.) Treacher-Collins syndrome and co-existing dermatomyositis.
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Author
Larenas-Linnemann DE; Berr´on-Perez R; Ortega-Martell JA; Onuma-Takane E; Huicochea-Grobet Z
Address
Instituto Nacional de Pediatria, Mexico City, Mexico.
Source
Ann Allergy Asthma Immunol, 80(1):50-4 1998 Jan

Abstract

BACKGROUND: Treacher-Collins syndrome, an autosomal dominantly inherited malformation of structures derived from the first and second branchial arch, has an incidence of 1:10,000 newborns. The prevalence of dermatomyositis at less than 24 years of age has been estimated at 1 per 100,000. The occurrence of both Treacher-Collins syndrome and dermatomyositis combined in the same patient should occur once in every 1,000,000,000 subjects.

METHODS: We report a patient with Treacher-Collins syndrome who developed dermatomyositis at the age of 5 years.

RESULTS: No other patient with both Treacher-Collins syndrome and an autoimmune disease has been reported. The thymus originates from the third branchial pouch and is unaffected by the syndrome. In Treacher-Collins syndrome the affected gene has been mapped to the fifth chromosome, while dermatomyositis is related to HLA B8 and DR3, coded on the sixth chromosome. No immunologic alteration has been described in patients with Treacher-Collins syndrome.

CONCLUSION: This is the first report of a patient with Treacher-Collins syndrome and dermatomyositis. There is no genetic or physiopathologic explanation for the concurrence of both conditions.

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33.) [A clinical analysis of cutaneous type dermatomyositis]
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Author
Yu B
Address
PUMC Hospital, CAMS, Beijing.
Source
Chung Kuo I Hsueh Ko Hsueh Yuan Hsueh Pao, 16(5):394-6 1994 Oct

Abstract

This paper reports nine patients with the classic cutaneous findings of dermatomyositis who did not develop clinical or laboratory evidence of muscle disease for at least 2 years after onset of their skin manifestations. Such patients represent 3.5% of our total experience with dermatomyositis patients during a 12 years period. None of the patients had evidence of malignancy.

Each of five patients treated with oral prednisone for their cutaneous lesion or mild myositis after onset of their skin manifestations 3-12 years and had marked improvement. The author emphasizes that the cutaneous manifestations of dermatomyositis are pathognomonic of this disease and the term of this disease proposes cutaneous type dermatomyositis better than amyopathic dermatomyositis.

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34.) Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis.
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Author
Requena L; Grilli R; Soriano L; Escalonilla P; Fari~na C; Mart´in L
Address
Department of Dermatology, Fundaci´on Jim´enez D´iaz, Universidad Aut´onoma, Madrid, Spain.
Source
Br J Dermatol, 136(5):768-71 1997 May

Abstract

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. These patients showed generalized follicular hyperkeratosis and diffuse thickening of the palms and soles. Histopathological findings consisted of keratotic plugging of the follicular infundibulum and features of erector pili myositis. We report on an 18-year-old woman with dermatomyositis.

The diagnosis was established by characteristic enzymatic alterations, electromyographic pattern of myositis and the findings in a muscle biopsy, although the patient had no evidence of muscular weakness during a follow-up of 14 years.

She developed an erythematosus and squamous eruption associated with diffuse palmoplantar keratoderma. Histopathological features consisted of a papillomatous epidermis with spicules of compact eosinophilic hyperkeratosis over the tips of papillae that were not related to hair follicles. Pityriasis rubra pilaris-like eruption seems to be a characteristic although uncommon cutaneous manifestation in dermatomyositis.

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35.) HCV and dermatomyositis: report of 5 cases of dermatomyositis in patients with HCV infection. ===================================================================== Author
Fiore G; Giacovazzo F; Giacovazzo M
Address
VI Medical Clinic, University La Sapienza, Rome, Italy.
Source
Riv Eur Sci Med Farmacol, 18(5-6):197-201 1996 Sep-Dec

Abstract

The authors report five cases of dermatomyositis in which positivity for hepatitis C virus was ascertained. A virus-related (ECHO virus, Coxsackie) dermatomyositis is known, but a hepatitis virus C-related dermatomyositis was reported only in one case in a Letter to the Editor of Journal of Rheumatology (1994).

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36.) [Dermatomyositis and cancer. A retrospective study]
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Author
Selvaag E; Thune P; Austad J
Address
Hudavdelingen Ullev al sykehus, Oslo.
Source
Tidsskr Nor Laegeforen, 114(20):2378-80 1994 Aug 30

Abstract

In a retrospective study, the files of 19 patients with dermatomyositis examined at our departments from 1970 to 1993 were reviewed. The parameters studied were age, sex, muscle enzyme values, muscle biopsies, electromyographical findings and interval from onset of dermatomyositis until first visit to the department. Out of 19 patients with dermatomyositis, 18 were adults and in nine of these the condition was associated with cancer (three out of three men, six out of 15 women).

Electromyographical findings were pathological in 17 investigated patients and myositis was indicated in 13 out of 15 biopsies. Muscle enzyme values were elevated in seven out of nine patients with cancer and in three out of nine without. Out of five patients with dysphagia, four patients had cancer.

The risk of cancer is increased in patients with dermatomyositis. Factors indicating a poor prognosis regarding the association between dermatomyositis and cancer in our study were old age, male sex and dysphagia.

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37.) Amyopathic dermatomyositis (dermatomyositis sin´e myositis). Presentation of six new cases and review of the literature [see comments]
=====================================================================
Author
Euwer RL; Sontheimer RD
Address
Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, TX 75235.
Source
J Am Acad Dermatol, 24(6 Pt 1):959-66 1991 Jun

Abstract

We report six patients with the classic cutaneous findings of dermatomyositis who did not develop clinical or laboratory evidence of muscle disease for at least 2 years after onset of their skin manifestations. Such patients represent 11% of our total experience with dermatomyositis patients during a 15 year period.

All six patients had Gottron's paules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. In addition, all complained of pruritus and photosensitivity. None of the patients had evidence of malignancy.

Each of five patients treated with oral corticosteroids for their cutaneous disease had marked improvement and did not develop myositis. These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic of this disease and challenge the commonly held notion that muscle disease always develops within 2 years of onset of skin disease.

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38.) Epstein-Barr virus strain type and latent membrane protein 1 gene deletions in lymphomas in patients with rheumatic diseases.
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Natkunam Y; Elenitoba-Johnson KS; Kingma DW; Kamel OW
Stanford University School of Medicine, California, USA.
Arthritis Rheum (UNITED STATES) Jun 1997 40 (6) p1152-6 ISSN: 0004-3591
Language: ENGLISH
Document Type: JOURNAL ARTICLE
Journal Announcement: 9708

Subfile: AIM; INDEX MEDICUS

OBJECTIVE: Recent studies have shown that immunomodulatory therapy for the treatment of rheumatic diseases can be associated with the development of Epstein- Barr virus (EBV)-associated lymphoproliferative disorders. The present study was undertaken to determine the strain type of EBV in lymphoproliferative disorders that occur in patients with rheumatic disease and to investigate EBV latent membrane protein 1 (LMP-1) gene deletions that occur in these lymphoproliferative disorders.

METHODS: Ten EBV-associated lymphoid neoplasms in patients with rheumatoid arthritis or dermatomyositis were analyzed by polymerase chain reaction to determine EBV strain type and to investigate for the presence of a previously characterized 30-basepair deletion in the LMP-1 gene.

RESULTS: The results indicated that lymphoproliferative disorders in these patients can harbor EBV strain type A or B, with a predominance of type A infection (80%). It was also shown that both wild-type and mutated LMP-1 genes can be found in these neoplasms, with the deleted form of the LMP-1 gene occurring in one-third of cases in this series.

CONCLUSION: LMP-1 deletions associated with certain aggressive lymphoid neoplasms are not required for the genesis of lymphoproliferative disorders in patients with rheumatic disease. The relative frequencies of type A and type B EBV strains in these lymphoproliferative disorders show similarities to the frequencies in patients with post-solid organ transplantation immunosuppression-associated lymphoproliferative disorders.

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DATA-MÉDICOS/DERMAGIC-EXPRESS No (52) 13/05/99 DR. JOSÉ LAPENTA R.
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