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Maximilian C. Aichelburg, MD; Robert Loewe, MD; Nikolaus Schicher, MD; Paul-Gunther Sator, MD; Franz M. Karlhofer, MD; Georg Stingl, MD; Ahmad Jalili, MD, PhD; Divisions of Immunology, Allergy, and Infectious Diseases (Drs Aichelburg, Karlhofer, Stingl, and Jalili) and General Dermatology (Drs Loewe and Schicher), Department of Dermatology, Medical University of Vienna, and Department of Dermatology, Hietzing Municipal Hospital (Dr Sator), Vienna, Austria

Background Scleredema adultorum of Buschke is an uncommon skin condition that may accompany diabetes mellitus. Current therapeutic options are poor, with only case reports and small series supporting their use. Observation We describe 3 patients with severe restrictive scleredema associated with type 2 diabetes mellitus who responded to radiation therapy after other treatments had failed. Angle of shoulder abduction was used as an objective measurement of improvement, while the patients rated several subjective areas, including arm range of motion, ease of performing daily tasks, ease of breathing, and neck thickening. Objective improvement tended to plateau over time but remained well above baseline. The duration of subjective improvement was variable, lasting from a few months to the end of the observation time of 2½ years. Conclusion Though not consistently durable in its effect, radiation therapy appears to be a viable therapeutic option in extreme cases of this difficult-to-treat disease.

Since the first description of scleredema adultorum by Buschke 1 in 1902, there have been numerous descriptions of this entity in the literature. The disease's special predilection for obese diabetics has also been noted. 2 We describe a case of scleredema adultorum in a diabetic whose course was progressive and punctuated by recurrent cellulitis in the region affected by scleredema. Report of a Case A 52-year-old diabetic woman was admitted to the University Hospital, Boston, on Sept 2, 1983, because of increasing pain and swelling in the skin on the back of her neck, as well as fever with shaking chills. In 1964 she had first noted the onset of "tightness" in her back following an episode of acute pharyngitis. During the next few years, the tightness, along with a woody induration, spread to involve the skin over the occiput, neck, interscapular areas, and lumbar region. Along with this slowly

• 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 (κ-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 proα1(1) 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. ( Arch Dermatol 1987;123:226-229)

• 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-λ 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. (Arch Dermatol1987;123:629-632)

• A 3-month-old infant with scleredema had associated cytomegalovirus pneumonia with a rapidly fatal course. Because differentiation of scleredema from sclerema neonatorum, subcutaneous fat necrosis, and scleroderma may be difficult, histologic diagnosis is important. Cetylpyridinium chloride was used successfully as a fixative to demonstrate acid mucopolysaccharides histologically. ( Arch Dermatol 1986;122:1417-1419)

• A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG - K and IgG 3 -K type. Only one of the three patients had IgG 1 -λ paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved. ( Arch Dermatol 1981;117:536-539)

This is a report of eight cases of scleredema associated with diabetes mellitus. There were six men and two women, with ages ranging from 41 to 80 years. The predominant clinical features were obesity, long-standing diabetes mellitus, and scleredema; there was a high incidence of heart disease. Five patients had diabetic retinopathy. Six were maturity-onset, overt diabetics while two were chemical or latent diabetics. Most patients were resistant to antidiabetic therapy. On skin biopsies the dermis was about three times thicker than normal. Histologically there was a marked, benign hyperplasia of collagen fibers involving the dermis and subcutaneous tissue. Chemical analysis of the skin revealed an increase in collagen and glycosaminoglycans, proportional to the increase in skin thickness. Analysis of the glycosaminoglycans revealed both hyaluronic acid and dermatan sulfate.

Eight cases of scleredema adultorum are presented, together with a review of 215 previously reported cases. Contrary to present day concepts, 25% of the cases showed either no improvement or only partial improvement two or more years after the onset of the disease. Unlike systemic sclerosis, with which it is often confused, scleredema adultorum frequently involves the tongue but not the esophagus, rarely the hands, and never the feet. A significant elevation of the anti-streptolysin 0 titer, which was associated with a preceding febrile illness, was observed in two patients in the pediatric age group, but not in any of the adult patients.

To the Editor.— Recently, an article entitled "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" by Oikarinen et al was published in theArchives. 1 The same patient that was in this study was also included in another study by Ohta et al 2 that was published in the Journal of the American Academy of Dermatology this January. In the last-mentioned article, four patients with scleredemalike skin changes and paraproteinemia were studied. Even though the same patient was included in both studies, the approach and methods of these studies were totally different. In the article by Oikarinen et al, the most important finding was that collagen synthesis and collagen messenger RNA were markedly elevated in the cell cultures from the fibrotic skin. However, the role of serum factors (ie, paraproteins) was not

Forty-four patients were investigated, 22 suffering from diffuse scleroderma and 22 from other clinically similar conditions. Horizontal sections were taken from biopsies, both from affected and healthy skin and stained with methylene blue. Histological findings were compared with clinical observations and laboratory data. The processes of degeneration and regeneration of the nerve network in diffuse scleroderma show some regularity and certain typical features within both the affected and apparently normal skin. On the contrary, in other conditions clinically resembling scleroderma no characteristic changes in the nerve network could be observed.