During May 1997-November 2000, eight (3%) of 265 kidney transplant recipients at a hospital in California developed an unusual skin condition posttransplant (Figure 1). On clinical examination, the patients had fibrotic skin lesions histologically resembling scleromyxedema on their distal extremities and trunk, resulting in severe contractions and limited mobility. However, the usual IgG lambda paraprotein associated with scleromyxedema was not observed in these patients. Personnel in the dermatopathology section at the University of California, San Francisco, reviewed the biopsies and concluded that this skin disorder had not been described previously. As a result, health-care providers at the hospital where the index patient was treated asked the California Department of Health Services (CD HS) and CDC to assist in the investigation. This report summarizes preliminary findings from the investigation.

A case was defined as large areas of hardened skin with slightly raised plaques or papules, with or without pigment alteration, in a patient with a skin biopsy indicating increased dermal fibroblasts and mucin and an abnormal dermal collagen bundle pattern. Additional patients were identified by responses to a publication describing the condition (1), by colleague referral, and by contacting members of the American Society of Dermatopathology, who were asked to alert other clinicians about the condition and to refer potential patients to CDHS. As of January 2002, 49 patients have been identified throughout the United States and Europe. Although having renal disease is not a part of the case definition, all patients have had underlying renal disease; approximately half have had renal transplantation. No consistently effective treatment exists; however, several patients have improved.