Scleredema adultorum of Buschke: a case report and review of the literature.

摘要

OBJECTIVES: To present a case of scleredema adultorum of Buschke associated with hypergammaglobulinemia and review the literature pertaining to this disease. METHODS: Search of MEDLINE (PubMed) was performed using the words "Scleredema Adultorum." Only cases in the English language, with pertinent clinical information for analysis, were included. RESULTS: We present a case of scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with scleredema. Our patient was found to have a B-cell lymphoma before being diagnosed with scleredema. The progression of skin thickening halted with no apparent correlation to immunosuppressive therapies or chemotherapeutic agents. A total of 165 cases of scleredema adultorum are described in the literature. There are 3 types of scleredema adultorum. Type 1 is usually preceded by a febrile episode and resolves spontaneously. Type 2 is associated with developing paraproteinemias including multiple myeloma. Type 3 is associated with diabetes mellitus. CONCLUSIONS: Scleredema adultorum of Buschke is a rare disorder which is sometimes associated with hypergammaglobulinemia; our patient had a B-cell lymphoma. Physicians should suspect scleredema in any patient with diffuse skin thickening where the hands and feet are spared, particularly if diabetes or a preceding febrile episode were present. Once the diagnosis of scleredema is made, evidence for the presence of hypergammaglobulinemia should be sought.