Therapy resistant idiopathic scleredema: an underlying pathology not always present.

Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of scleredema which the diagnosis didn't get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease.