Background: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality.
Objective: Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents.
Methods: A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted.
Results: Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients.
Conclusion: The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.