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Scleredema adultorum is a rare sclerotic disorder characterized by diffuse swelling and nonpitting induration of the skin. Its occurrence has been documented in association with infections, diabetes mellitus, paraproteinemia, multiple myeloma, and monoclonal gammopathy. We report an unusual case of a 48-year-old man with an asymptomatic bilateral eyelid edema of sudden onset. During a period of 6 months, the condition slowly progressed to extensive nonpitting edematous swelling restricted to the periorbital sites. The presumptive diagnosis of scleredema adultorum was confirmed by the presence of typical histologic findings. This case is unique in that the periorbital swelling remained as the sole clinical manifestation of scleredema during the 5-year follow-up and was complicated with partial vision blockage.

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.

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/cm 2 ). Ultrasonography showed significant reduction in both skin thickness and density. Conclusion: Bath-PUVA therapy appears to be effective in the treatment of SA.(J Am Acad Dermatol 1998;38:240-2.)

This report reflects the best data available at the time the report was prepared, but caution should be exercised in interpreting the data; the results of future studies may require alteration of the conclusions or recommendations set forth in this report .

A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.

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.