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****** DATA-MÉDICOS **********
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ESCLEREDEMA DE BUSCHKE
SCLEREDEMA OF BUSCHKE
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****** DERMAGIC-EXPRESS No.60 ******* 
****** 30 JUNIO DE 1.999 *********** 
30 JUNE 1.999
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EDITORIAL ESPAÑOL:
=====================
Hola amigos de la red, en esta ooportunidad DERMAGIC les presenta una pequeña revisión de la enfermedad: ESCLEREDEMA DE BUSCHKE.

Esta patología esta  asociada a Diabetes Mellitus. La semana pasada fue requerida en la la lista DERMLIST de Brasil. Espero que estas 32 referencias nos ilustren bien el tema.

En el attach una lamina ilustrativa: Escleredema de Buschke, cuello y hombros.

saludos a todos !!!

Dr. José Lapenta R.,,,


EDITORIAL ENGLISH:
=====================
Hello friends of the net, in this opportunity DERMAGIC presents a short review of the illness: SCLEREDEMA OF BUSCHKE,

This pathology is associated to Diabetes Mellitus. Last week it was required in the the list DERMLIST of Brazil. I hope these 32 references illustrate us well the topic. 

In the attach an illustrative sheet: Scleredema of Buschke, neck and shoulders. 

Greetings to ALL, !!

Dr. José Lapenta R.,,

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DERMAGIC/EXPRESS(60)
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ESCLEREDEMA DE BUSCHKE  / SCLEREDEMA OF BUSCHKE 
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1.) Scleredema of Buschke: a report of seven cases. 
2.) In vivo study of skin mechanical properties in scleredema of Buschke. 
3.) Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia. 
4.) Scleredema of Buschke successfully treated with electron beam therapy. 
5.) Scleroderma-like disorders. 
6.) [Scleredema adultorum Buschke. Case report and review of the literature] 
7.) Scleredema diabeticorum of Buschke confined to the thighs. 
8.) [Scleredema, acanthosis nigricans and IgA/Kappa multiple myeloma] 
9.) Bath-PUVA therapy in three patients with scleredema adultorum. 
10.) Diabetic scleredema: a case report and biochemical analysis for glycosaminoglycans. 
11.) Severe diabetic scleredema with extension to the extremities and effective treatment using prostaglandin E1. 
12.) [Systemic Buschke's scleredema with cardiomyopathy, monoclonal IgG kappa gammopathy and amyloidosis. Case report with autopsy].
13.) Demonstration of increased levels of type I collagen mRNA using quantitative polymerase chain reaction in fibrotic and granulomatous skin diseases.
14.) Increased collagen propeptides in the skin of a scleredema patient but no change in re-epithelialisation rate.
15.) Scleroedema in a child.
16.) Biochemical characterization and tissue distribution of the scleredema in a case of Buschke's disease.
17.) Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro.
18.) Scleredema revisited. A poststreptococcal complication.
19.) A fatal case of scleredema of Buschke.
20.) Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia.
21.) Diabetic scleredema and scleroderma-like changes in a patient with maturity onset type diabetes of young people.
22.) Scleredema and smoldering myeloma.
23.) Acute scleredema with fatal outcome.
24.) Acid mucopolysaccharide staining in scleredema.
25.) Scleredema of Buschke associated with rheumatoid arthritis and Sjogren's syndrome.
26.) [Post-traumatic scleroedema adultorum].
27.) Scleroedema diabeticorum: a minor but often unrecognized complication of diabetes mellitus.
28.) Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum.
29.) Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation.
30.) Scleredema and paraproteinemia. Enhanced collagen production and elevated type I procollagen messenger RNA level in fibroblasts grown from cultures from the fibrotic skin of a patient.
31.) Paraproteinemia in patients with scleredema. Clinical findings and serum effects on skin fibroblasts in vitro.
32.) Biochemical characterization and tissue distribution of the scleredema in a case of Buschke's disease.
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1.) Scleredema of Buschke: a report of seven cases. 
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Author 
Tate BJ; Kelly JW; Rotstein H 
Address 
Dermatology Unit, Alfred Hospital, Prahran, Vic, Australia. 
Source 
Australas J Dermatol, 37(3):139-42 1996 Aug 

Abstract 

Scleredema of Buschke is an uncommon dermatosis characterized by thickened, indurated skin, sometimes with erythema. Histopathology shows thickened dermal collagen with a mild infiltrate of mucin in the deeper dermis. Seven adults with scleredema are presented, four females and three males, and their mean age at diagnosis was 54 years. All had diabetes mellitus for an average of 13 years prior to the onset of scleredema.

Complications of diabetes, especially retinopathy (n = 5), neuropathy (n = 4) and peripheral vascular disease (n = 3), were present in five patients. One patient died (cause not established), and another has life-threatening cardiomyopathy. We have no evidence that the scleredema was a significant aetiologic factor in either case, despite published reports of fatalities related to scleredema. Three patients were followed up for more than 1 year and, irrespective of therapy, the scleredema did not resolve in any patient. 

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2.) In vivo study of skin mechanical properties in scleredema of Buschke. 
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Author Dobrev H 
Address 
Department of Dermatology, Medical University, Plovdiv, Bulgaria. 
Source 
Acta Derm Venereol, 78(2):103-6 1998 Mar 

Abstract 

A non-invasive, in vivo suction device was used to investigate the mechanical properties of the skin in a patient with scleredema of Buschke.

Clinical scoring of skin induration and measurements of skin elasticity were performed over 9 anatomic regions on admission and after 3 (on discharge), 17 and 28 months. Immediate distension, final distension and immediate retraction were significantly decreased, while the viscoelastic to elastic ratio was significantly increased in the patient as compared to the healthy controls.

Delayed distension and biological elasticity were preserved. Low values of skin distensibility correlated with a severe skin induration (p < 0.001). The changes were more expressive with the 8 mm-diameter measuring probe than the 2 mm-diameter probe. The method applied can be used for objective and quantitative assessment of skin involvement in scleredema of Buschke. 

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3.) Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia. 
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Author 
Basarab T; Burrows NP; Munn SE; Russell Jones R 
Address 
Department of Dermatology, Ealing Hospital, Southall, Middlesex, UK. 
Source 
Br J Dermatol, 136(6):939-42 1997 Jun 

Abstract 

Scleredema is a rare primary cutaneous mucinosis. Systemic involvement is uncommon and histological confirmation is often lacking. We report a case of a 60-year-old man with scleredema and evidence of mucin deposition on biopsies from multiple extracutaneous sites. The bone marrow, nerve, hepatic and salivary gland involvement seen on histology in our patient has not, to our knowledge, been previously reported in this condition. 

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4.) Scleredema of Buschke successfully treated with electron beam therapy. 
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Author 

Tamburin LM; Pena JR; Meredith R; Soong VY 
Address 
Department of Dermatology, University of Alabama at Birmingham, USA. 
Source 
Arch Dermatol, 134(4):419-22 1998 Apr 
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5.) Scleroderma-like disorders. 
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Author 
Jablonska S; Blaszczyk M 
Address 
Department of Dermatology, Warsaw School of Medicine, Poland. 
Source 
Semin Cutan Med Surg, 17(1):65-76 1998 Mar 

Abstract 

Scleroderma-like disorders are widely disparate conditions mimicking either systemic sclerosis or cutaneous localized scleroderma, not infrequently displaying features of both. Some are exclusively sclerotic, some scleroatrophic with prevailing sclerosis or atrophies.

The recognition of scleroderma-like disorders is of practical importance because by establishing the cause of the disease, it is possible to introduce an effective therapy, as in scleredema Buschke or scleredema diabeticorum, sclerodermiform porphyria, Borrelia burgdorferi-induced sclerodermiform acrodermatitis atrophicans, sclerodermiform phenylketonuria, drug-induced conditions, and so on. Scleroderma-like disorders strongly suggest that the pathogenesis of skin sclerosis and internal involvement may be divergent, and of various causes.

Some of them, such as atrophoderma Pasini-Pierini or progressive facial hemiatrophy, frequently overlapping with scleroderma, make the differentiation very difficult, if at all possible, and the diagnosis is often arbitrary. Some, as sclerodermiform graft-versus-host reaction, point to the autoimmune origin of scleroderma.

The amply-covered congenital sclerodermiform conditions present a large spectrum of still not widely known and extremely heterogeneous syndromes, associated with numerous anomalies and/or malignancies. 

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6.) [Scleredema adultorum Buschke. Case report and review of the literature] 
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Author 
Ulmer A; Schaumburg-Lever G; Bauer J; K¨otter I; Fierlbeck G 
Address 
Universit¨ats-Hautklinik T¨ubingen. 
Source 
Hautarzt, 49(1):48-54 1998 Jan 

Abstract 

Scleredema adultorum of Buschke is a rare disorder of unknown aetiology. It is characterized by diffuse, non-pitting swelling and induration of the skin.

Skin biopsies reveal marked thickening of the dermis due to collagenous replacement of the subcutis and deposition of hyaluronic acid between the collagen fibers. The disease classically only affects the skin. In 24 cases an associated monoclonal gammopathy has been detected.

A 75-year-old patient had a 19-year history of scleredema adultorum. In addition to a monoclonal gammopathy the patient suffered from involvement of the tongue, pharynx and upper esophagus. Furthermore a polyneuropathy, ocular involvement with restricted eye movements and a sicca syndrome were present.

The simultaneous occurrence of cutaneous scleredema with any one of the above mentioned symptoms has been reported before. The wide variety of extracutaneous manifestations of scleredema as found in our patient is amazing and has not been previously described. 

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7.) Scleredema diabeticorum of Buschke confined to the thighs. 
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Author 
Farrell AM; Branfoot AC; Moss J; Papadaki L; Woodrow DF; Bunker CB 
Address 
Department of Dermatology, Charing Cross and Westminster Medical School, London, U.K. 
Source 
Br J Dermatol, 134(6):1113-5 1996 Jun 

Abstract 

We describe a patient with insulin-dependent diabetes without vascular complications in whom scleredema was confined to the thighs. Electron microscopy demonstrated heterogeneity in both the size and density of the collagen fibrils and the presence of filamentous material between them. 

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8.) [Scleredema, acanthosis nigricans and IgA/Kappa multiple myeloma] 
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Author 
Valente L; Velho GC; Farinha F; Bernardo A; Ribeiro P; Massa A 
Address 
Service de M´edecine, hospital Geral de Santo Ant´onio, Porto, Portugal. 
Source 
Ann Dermatol Venereol, 124(8):537-9 1997 

Abstract 

BACKGROUND: Scleredema is an uncommon disease of unknown origin. Characteristic thick skin with symmetrical diffuse induration develops. The infiltration begins on the face and neck then extends to the root of the upper limbs and trunk. There are three clinical types of scleredema.

The first is preceded by an upper airway infection and progresses rapidly before regressing spontaneously within a few months. The second type is associated with chronic diabetes. The third type is associated with monoclonal gammapathy, rarely of myelomatous type, and develops insidiously. Acanthosis nigricans can be a paraneoplastic syndrome, often associated with a gastrointestinal cancer. Few cases associating scleredema and acanathosis nigricans have been reported.

CASE REPORT: A 56-year old woman had developed scleredema over the last 6 years when acanthosis nigricans appeared together with IgA kappa multiple myeloma. Treatment with melphalan and prednisolone was effective against the myeloma as well as the scleredema and acanthosis nigricans.

Discussion: Only five cases of associated scleredema and multiple myeloma have been reported, four with kappa IgG myeloma and one with IgA myeloma. An association between acanthosis nigricans and sclerederma could be coincidental although the fact that the different manifestations regressed together after the myeloma treatment would suggest some relationship between these three diseases. 

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9.) Bath-PUVA therapy in three patients with scleredema adultorum. 
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Author 
Hager CM; Sobhi HA; Hunzelmann N; Wickenhauser C; Scharenberg R; Krieg T; Scharffetter-Kochanek K 
Address 
Department of Dermatology, University of Cologne, Germany. 
Source 
J Am Acad Dermatol, 38(2 Pt 1):240-2 1998 Feb 

Abstract 

BACKGROUND: Scleredema adultorum (SA) is a rare connective tissue disorder for which no treatment has proven to be effective.

OBJECTIVE: Our purpose was to determine the effect of bath-PUVA therapy on SA. METHODS: Three patients were treated. Clinical evaluation of skin induration and thickness as well as ultrasonography were performed at baseline and after treatment.

RESULTS: All three patients showed substantial clinical improvement with bath-PUVA therapy (median of 59 treatments and a cumulative UVA dose of 245.7 J/cm2). Ultrasonography showed significant reduction in both skin thickness and density.

CONCLUSION: Bath-PUVA therapy appears to be effective in the treatment of SA. 

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10.) Diabetic scleredema: a case report and biochemical analysis for glycosaminoglycans. 
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Author 
Kobayashi T; Yamasaki Y; Watanabe T 
Address 
Division of Dermatology, Tokyo Daini National Hospital, Higashigaoka, Meguro, Japan. 
Source 
J Dermatol, 24(2):100-3 1997 Feb 

Abstract 

We report a patient with the typical lesions of diabetic scleredema. Histological findings of the involved skin were thickening of the dermis, depositions of mucins, and fibrosis. Biochemical analysis revealed an increase in glycosaminoglycans in the involved skin as well as in the cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic acid. 

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11.) Severe diabetic scleredema with extension to the extremities and effective treatment using prostaglandin E1. 
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Author 
Ikeda Y; Suehiro T; Abe T; Yoshida T; Shinoki T; Tahara K; Nishiyama M; Okabayashi T; Nakamura T; Itoh H; Hashimoto K 
Address 
Second Department of Internal Medicine, Kochi Medical School, Nankoku. 
Source 
Intern Med, 37(10):861-4 1998 Oct 

Abstract 

We report a 49-year-old woman with severe diabetic scleredema (DS). The patient had non-insulin-dependent diabetes mellitus (NIDDM) for 9 years and noticed thickened skin on her back 3 years previously. Her DS rapidly extended to her back and extremities with pain and immobility.

Her symptoms of DS improved dramatically after establishing strict glycemic control and intravenous administration of prostaglandin E1 (PGE1). However, the histological findings of her skin biopsy did not change even after the treatment for 12 weeks, and her symptoms worsened again after discontinuation of glycemic control and PGE1 treatment.

The causes of DS have been considered to be metabolic abnormalities associated with hyperglycemia and hypoxia in the skin due to diabetic microangiopathy. PGE1 was an effective treatment for DS in our patient. Strict control of hyperglycemia and PGE1 treatment may be sufficient to manage DS, although a very long treatment period is necessary. 

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12.) [Systemic Buschke's scleredema with cardiomyopathy, monoclonal IgG kappa gammopathy and amyloidosis. Case report with autopsy].
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Medicina (B Aires) 1998;58(5 Pt 1):501-3 

Paz RA, Badra RE, Marti HM, Maxit MJ
Hospital Privado de Comunidad, Mar del Plata, Argentina.
[email protected] 

A 73 year old retired truck driver and blacksmith was studied in June 1996 for thoracic pain and was diagnosed as acute pericarditis which responded well to steroid treatment. In January 1997, he noted swelling of the abdominal skin, genitalia and limbs, sparing the feet. He was euthyroid, did not have evidence of diabetes or a Raynaud's phenomenon.

His proteinogram showed an IgG-Kappa monoclonal paraprotein M component, 1.31 g/oo. TSH and tetraiodotironine were normal; ESR 16 mm in the first hour. As he did not respond to treatment he was referred to our hospital in March 1997. On physical examination the most relevant findings were a non-pitting edema of the abdomen and lower limbs, sparing the feet.

An echocardiogram was consistent with an infiltrative cardiomyopathy. Soon after his hospitalization his condition worsened suddenly with severe bradicardia (28/minute) due to a junctional rhythm and righ bundle branch block. He suffered a cardiac arrest and died. The autopsy findings favoured the diagnosis of systemic scleredema adultorum of Buschke.

Amyloid deposits were also found although not abundant, with a similar distribution except in the skin. In this article the clinical and autopsy findings are presented in a patient showing coexistence of systemic Buschke's scleredema with an infiltrative cardiomyopathy, IgG Kappa gammopathy and amyloidosis. 

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13.) Demonstration of increased levels of type I collagen mRNA using quantitative polymerase chain reaction in fibrotic and granulomatous skin diseases.
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Br J Dermatol 1998 Jul;139(1):23-6 

Tasanen K, Palatsi R, Oikarinen A
Department of Dermatology, Oulu University Hospital, Finland. 

Collagen changes occur in localized scleroderma, scleredema and sarcoidosis. Previous biochemical, immunohistochemical and in situ hybridization studies have revealed increased collagen synthesis in these diseases. In the present study, we measured by pro alpha 1 (I) collagen and beta-actin mRNA levels in skin punch biopsy specimens from lesional and healthy skin using a quantitative polymerase chain reaction (PCR).

In this method, the targeted mRNA and a synthetic RNA as a internal standard are co-amplified together with the same primers. The amount of pro alpha 1 (I) collagen mRNA in cutaneous sarcoidosis lesions was found to be increased about two- to threefold compared with the values obtained for the healthy skin of the same two patients. In lesional skin of three patients with localized scleroderma the number of pro alpha 1 (I) collagen molecules was increased about two-fold.

The beta-actin mRNA values were at the same level in the affected and unaffected skin of all the patients studied. In conclusion, a marked increase in type I collagen gene expression was seen in localized scleroderma and scleredema, leading to fibrosis of the skin, and in a granulomatous skin disease, cutaneous sarcoidosis. 

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14.) Increased collagen propeptides in the skin of a scleredema patient but
no change in re-epithelialisation rate.
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Acta Derm Venereol 1996 Jul;76(4):305-9 

Haapasaari KM, Kallioinen M, Tasanen K, Risteli J, Palatsi R, Oikarinen A
Department of Dermatology, University of Oulu, Finland. 

Scleredema is a rare disease, affecting the skin connective tissue with increased amounts of collagen and glycosaminoglycans.

In the present study, the collagen synthesis and re-epithelialisation rate were measured from a 64-year-old male patient, who rapidly developed extensive tightening of the skin, without any underlying disease. The skin was thickened at several sites when measured with ultrasound, and the histology revealed accumulation of glycosaminoglycans and collagen bundles.

The collagen synthesis rate was measured from suction blisters induced on two different sites of the skin before the treatment and three times later up to 6 months after the treatment with a systemic steroid was started. The aminoterminal propeptide of type I collagen (PINP) was increased manifold in the affected skin when compared with the controls, indicating active collagen deposition in vivo.

Systemic steroid medication with high doses (over 20 mg/d) decreased both the type I and the type III collagen propeptide levels. The time schedule of the decreases in the propeptides in the thickened, affected skin and in the clinically normal-looking skin varied, and especially in the thickened skin in the abdomen the decrease in PINP was noted only after 3 months of prednisolone therapy.

When the prednisolone dose was only 10 mg daily, the propeptides were again up-regulated, perhaps reflecting the natural course of the disease. The re-epithelialisation rates at two different sites of the patient were similar to those in the controls, suggesting that even massive fibrosis with active deposition of collagen does not alter the basal rate of re-epithelialisation in the skin. In conclusion, collagen synthesis is markedly elevated in scleredema, leading to fibrosis of the skin.

A recently developed method utilizing assays of collagen propeptides from suction blister fluid allows monitoring of the collagen synthesis and detection of changes in the collagen synthesis during the treatment of fibrotic disorders. 

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15.) Scleroedema in a child.
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J Dermatol 1996 Jul;23(7):495-8 

Mitsuhashi Y, Kondo S, Shimizu Y
Department of Dermatology, Yamagata University School of Medicine, Japan. 

A 3-year-old Japanese girl with scleroedema was reported. She had had no signs of diabetes but did have a preceding bacterial infection in the tonsils three weeks before the skin lesion appeared.

The skin on the face, shoulders, extensor aspect of the upper arms, and proximal half of the forearms was indurated. The skin lesions expanded from the middle part of the forearms to the wrists during the observation period. Thereafter, the induration gradually disappeared.

A literature review revealed there were only six reports of scleroedema in children under 15 years old before 1996 in Japan; a total of 166 cases of the disease was reported in the same period. Five out of these six cases were not diabetes-associated. All but one of these six patients were female. Juvenile scleroedema seems to be rare in Japan. 

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16.) Biochemical characterization and tissue distribution of the scleredema in a case of Buschke's disease.
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Acta Derm Venereol 1987;67(3):193-8 

Roupe G, Laurent TC, Malmstrom A, Suurkula M, Sarnstrand B

Biopsies from a patient with a longstanding form of scleredema adultorum Buschke were analysed for morphological and biochemical changes in the dermal connective tissue. By light microscopy the tissue changes were located to the deep part of the reticular dermis.

Therefore dermal tissue was separated into a superficial and a deep part and analysed biochemically. By this procedure it was possible to show that the concentration of hyaluronan in the deep part of the dermis was increased. The urinary excretion of methylimidazole acetic acid, an indicator of the mast cell mass in the body, was also elevated. 

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17.) Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro.
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Br J Dermatol 1995 Jun;132(6):992-9 

Varga J, Gotta S, Li L, Sollberg S, Di Leonardo M
Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA 19107, USA. 

To elucidate the mechanisms involved in the development of cutaneous fibrosis in scleredema adultorum, we studied a patient with long-standing scleredema who had no history of diabetes mellitus or preceding febrile illness.

Histological examination of a biopsy specimen from involved forearm skin demonstrated marked thickening of the dermis and accumulation of mucin between collagen bundles. Increased levels of type I collagen mRNA, as evidenced by positive in situ hybridization signals with an alpha 1(I) procollagen cDNA were found in numerous fibroblasts throughout the dermis.

The expression of several genes coding for proteins involved in the maintenance of connective tissue was examined by determining in vitro protein production and mRNA levels in fibroblasts from the affected skin. Total protein production, glucosamine incorporation and collagen synthesis, were elevated by 44-97% in scleredema fibroblasts, compared with fibroblasts from two healthy individuals. Levels of mRNAs for alpha 1(I) and alpha 1(III) procollagens and fibronectin were elevated in scleredema fibroblasts, whereas mRNA levels for the tissue inhibitor of metalloproteinase were unaltered compared with control cultures.

The results suggest that fibroblasts from the involved skin in non-diabetic patients with scleredema may exhibit a biosynthetically activated phenotype, which persists for several years. These alterations are likely to be involved in the development of the cutaneous induration and thickening which is characteristic of this disease. 

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18.) Scleredema revisited. A poststreptococcal complication.
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Clin Pediatr (Phila) 1994 Oct;33(10):606-10 

Cron RQ, Swetter SM
Department of Pediatrics, Lucille Salter Packard Children's Hospital at Stanford, California. 

Scleredema is a rare connective disease which must be differentiated from scleroderma in childhood. Scleredema is characterized by thickening of the dermis of the neck, head, and upper trunk. We report a case of scleredema in an 8-year-old boy with coincident streptococcal colonization.

The patient report demonstrates many of the common features of scleredema, including an associated streptococcal infection, a relatively benign presentation of illness, and the characteristic mucopolysaccharide intradermal staining on skin biopsy.

The literature on scleredema is reviewed, focusing on the disease course, differential diagnosis, and an overview of the proposed three subgroups of scleredema. The association of scleredema to a prior streptococcal infection is explored, and a proposed autoimmune pathophysiology of the disease, as it relates to streptococcal infection, is presented. 

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19.) A fatal case of scleredema of Buschke.
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Br J Dermatol 1994 May;130(5):669-70 

Sansom JE, Sheehan AL, Kennedy CT, Delaney TJ
Department of Dermatology, Bristol Royal Infirmary, U.K. 

Scleredema of Buschke is a rare disorder characterized by the development of areas of skin induration which usually resolve spontaneously. It is occasionally associated with a benign gammopathy, and rarely with myelomatosis.

We describe a 60-year-old woman with extensive skin changes, who developed IgA myeloma. Unusually, her skin disease did not respond to conventional myeloma therapy. Death occurred as a consequence of the progressive skin disease. 

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20.) Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia.
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Br J Dermatol 1994 Mar;130(3):394-7 

Angeli-Besson C, Koeppel MC, Jacquet P, Andrac L, Sayag J
Department of Dermatology, CHU la Timone, Marseille, France. 

We report a case of scleredema adultorum (Buschke's disease) associated with an IgA kappa monoclonal hypergammaglobulinaemia. A significant improvement in the skin was obtained with electron-beam therapy. Scleredema would appear to be linked to monoclonal hypergammaglobulinaemia, but the relationship between the skin disorder and the immunoglobulin abnormality remains to be elucidated. 

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21.) Diabetic scleredema and scleroderma-like changes in a patient with maturity onset type diabetes of young people.
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Dermatology 1994;188(3):228-31 

Iwasaki T, Kohama T, Houjou S, Iriuchijima T, Ohtsuka T, Ishikawa T, Yamakage A, Mori M
Department of Internal Medicine, University School of Medicine, Maebashi, Japan. 

A 21-year-old housewife with maturity onset type diabetes of young people developed scleredema diabeticorum, scleroderma-like skin thickness on forearms and dorsum of hands, digital sclerosis and cheiroarthropathy. She had diabetes mellitus since the age of 11 years. Her grandfather on the mother's side, her mother and 3 of 5 her mother's brothers and sisters have diabetes mellitus.

Blood glucose was 295 mg/dl. Urinary glucose was 5.3 g/day. Nail fold capillary microscopy revealed a progressive systemic sclerosis pattern. Histologically, hematoxylin and eosin sections from back and forearm skin demonstrated broad collagen bundles separated by widened clear spaces throughout the thickened dermis. 

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22.) Scleredema and smoldering myeloma.
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J Am Acad Dermatol 1992 Feb;26(2 Pt 2):319-21 

Schmidt KT, Gattuso P, Messmore H, Shrit MA, Massa M, Welykyj S
Department of Dermatology, Loyola University Medical Center, Maywood, IL 60153. 

A 46-year-old white man had classic findings of scleredema; subsequently, a monoclonal immunoglobulin G lambda light chain was detected in his serum. Findings of a bone marrow biopsy specimen revealed that he had an increased percentage of plasma cells, some of which were atypical. A diagnosis of scleredema and smoldering myeloma was made. 

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23.) Acute scleredema with fatal outcome.
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J Assoc Physicians India 1990 Oct;38(10):807-8 

Shah PK, Lakhotia M, Jain SK, Meena RC
Department of Internal Medicine, Dr SN Medical College, Hospitals, Jodhpur. 

Death due to scleredema is rare. We report a patient with scleredema, which had an acute onset and rapid progression, leading to death within a month. Such an acute course terminating in fatality has not been described earlier. 

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24.) Acid mucopolysaccharide staining in scleredema.
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J Cutan Pathol 1990 Aug;17(4):211-3 

Cole HG, Winkelmann RK
Department of Dermatology, Mayo Clinic, Rochester, MN 55905. 

We studied 26 formalin-fixed biopsy specimens taken from 22 patients with scleredema and found positive staining in 67% of the tissues when both colloidal iron and alcian blue methods were used. Single stains were positive in only half the tissues.

Positive findings were noted in both patients with diabetes (11 patients) and in those without it (11 patients).

Our experience indicates that multiple biopsies may be necessary to demonstrate mucin in the dermis. Specimens obtained by incisional biopsy are preferred. 

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25.) Scleredema of Buschke associated with rheumatoid arthritis and Sjogren's syndrome.
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Br J Dermatol 1989 Oct;121(4):517-20 

Miyagawa S, Dohi K, Tsuruta S, Shirai T
Department of Dermatology, Nara Medical University, Japan. 

A case of scleredema of Buschke associated with rheumatoid arthritis and Sjogren's syndrome is described. The onset of the skin changes and rheumatoid arthritis was almost simultaneous and the sicca syndrome developed 4 years later. 

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26.) [Post-traumatic scleroedema adultorum].
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Derm Beruf Umwelt 1989 Sep-Oct;37(5):176-8 

Bergner T, Przybilla B
Dermatologischen Klinik und Poliklinik, Ludwig-Maximilians-Universitat Munchen. 

A 48-year-old male developed an induration of the skin of the neck a few days after a blunt trauma to this region had occurred. A diagnosis of scleredema adultorum (Buschke) could be established 3 years later. In view of the history and the lack of other diseases thought to be related to the elicitation of scleredema, a traumatic origin of the skin condition is probable. 

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27.) Scleroedema diabeticorum: a minor but often unrecognized complication
of diabetes mellitus.
===================================================================
Diabet Med 1988 Jul-Aug;5(5):465-8 

Sattar MA, Diab S, Sugathan TN, Sivanandasingham P, Fenech FF
Department of Medicine, Faculty of Medicine, Kuwait University, Safat. 

The association of specific dermatological complications with diabetes mellitus is well recognized. Of 100 hospital-based patients with diabetes mellitus (age 48 years +/- 2SE), 14% had scleroedema diabeticorum.

The affected subjects had a higher prevalence of retinopathy (p less than 0.001) and albuminuria (p less than 0.025). The duration of scleroedema correlated with the duration of diabetes (p less than 0.005). These findings highlight the relatively common occurrence of this skin condition which often goes unrecognized in people with diabetes. 

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28.) Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum.
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Arch Intern Med 1988 Mar;148(3):551-3 

Rimon D, Lurie M, Storch S, Halon D, Eisenkraft S, Laor A, Cohen L
Department of Medicine B, Carmel Hospital, Haifa, Israel. 

Multiple myeloma and congestive heart failure developed in a patient with long-standing scleredema adultorum. Staining of the myocardium, performed after her death, was positive for acid mucopolysaccharide and negative for amyloid. To the best of our knowledge, this is the first case in which acid mucopolysaccharide has been demonstrated in the myocardium, thus explaining the cardiomyopathy of scleredema adultorum.

Review of the world literature enabled us to identify a statistically significant increased prevalence of plasma cell dyscrasia among patients with protracted scleredema. In all patients, plasma cell dyscrasia appeared years after the onset of scleredema. Immunofluorescent studies were negative for immunoglobulin deposition.

We assume, therefore, that the plasma cell dyscrasia was secondary to scleredema. 

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29.) Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation.
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Arch Dermatol 1987 May;123(5):629-32 

McFadden N, Ree K, Soyland E, Larsen TE

Scleredema associated with a monoclonal gammopathy and generalized skin pigmentation is described in a 56-year-old man with hyperlipoproteinemia and cardiovascular disease.

The patient had IgG-lambda paraproteinemia, without any evidence of multiple myeloma or immunoglobulin deposition in affected skin. Ultrastructural studies of pigmented lesional skin showed increased transfer of melanosomes to basal keratinocytes and dermal melanophages containing complex melanosomes.

 In addition, cytoplasmic, electron-opaque lipid droplets were seen in approximately every third keratinocyte or melanocyte, while only an occasional dermal cell contained lipid droplets. The hyperpigmentation appeared to be directly related to the scleredema, while the lipid deposition in skin was a likely consequence of the hyperlipoproteinemia.

The findings further support the contention that paraproteinemia and hyperpigmentation may, in some patients, be associated features of scleredema adultorum. 

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30.) Scleredema and paraproteinemia. Enhanced collagen production and elevated type I procollagen messenger RNA level in fibroblasts grown from cultures from the fibrotic skin of a patient.
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Arch Dermatol 1987 Feb;123(2):226-9 

Oikarinen A, Ala-Kokko L, Palatsi R, Peltonen L, Uitto J

An edematous rash developed on the abdominal skin of a 76-year-old woman who had had diabetes mellitus for ten years. Some months later, the affected skin became thickened and indurated. Histopathologic examination revealed marked dermal fibrosis with excessive deposition of collagen.

The patient also had IgA (k-type) paraproteinemia. Fibroblast cultures from the affected and unaffected skin were studied for collagen metabolism. Procollagen synthesis was elevated about sixfold on fibroblasts derived from the affected skin.

A similar increase was detected in messenger RNA (mRNA) levels using a complementary DNA clone specific for human pro alpha 1(l) collagen mRNA. The elevated mRNA level could be the result of increased transcriptional activity of collagen genes or decreased degradation of collagen mRNAs.

Our findings suggest that increased collagen deposition may account for the marked dermal fibrosis that we observed in this patient. 

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31.) Paraproteinemia in patients with scleredema. Clinical findings and serum effects on skin fibroblasts in vitro.
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J Am Acad Dermatol 1987 Jan;16(1 Pt 1):96-107 

Ohta A, Uitto J, Oikarinen AI, Palatsi R, Mitrane M, Bancila EA, Seibold JR, Kim HC

Four patients with paraproteinemia and scleredema were studied. Histologic features included marked thickening and fibrosis of the dermis and subcutis.

Variable amounts of mucin deposits were detected in the interfibrillar spaces. Serum from one patient significantly stimulated collagen production in normal skin fibroblast cultures, whereas serum from another patient stimulated collagen production in autologous cell cultures.

Moreover, serum from one patient stimulated the [35S]sulfate incorporation into the fibroblasts. Circulating serum factors, possibly related to the paraprotein, may enhance the synthesis of extracellular macromolecules by dermal fibroblasts in these patients, thus providing a mechanism for dermal fibrosis. 

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32.) Biochemical characterization and tissue distribution of the scleredema in a case of Buschke's disease.
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Acta Derm Venereol 1987;67(3):193-8 

Roupe G, Laurent TC, Malmstrom A, Suurkula M, Sarnstrand B

Biopsies from a patient with a longstanding form of scleredema adultorum Buschke were analysed for morphological and biochemical changes in the dermal connective tissue. By light microscopy the tissue changes were located to the deep part of the reticular dermis.

Therefore dermal tissue was separated into a superficial and a deep part and analysed biochemically. By this procedure it was possible to show that the concentration of hyaluronan in the deep part of the dermis was increased. The urinary excretion of methylimidazole acetic acid, an indicator of the mast cell mass in the body, was also elevated. 

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DATA-MEDICOS/DERMAGIC-EXPRESS No (63) 30/06/99 DR. JOSE LAPENTA R. 
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