Scleredema adultorum of Buschke: a clinical, pathologic, and cell culture study of two patients.

Two patients with scleredema of Buschke are presented. The second patient developed scleredema after a febrile drug reaction. Biopsies of both involved and uninvolved skin were obtained for histologic examination and cell culture studies. Immunofluorescent studies of the biopsy specimens revealed staining with IgG, IgM and C3 at the dermal-epidermal junction in the involved skin of the first patient. This has not been previously reported. Cell culture studies revealed that fibroblasts from the involved skin produced more glycosaminoglycans (GAG) than uninvolved skin and most of the GAG produced was hyaluronic acid. Possible pathogenic mechanisms for this unusual condition are discussed.