Nodular scleroderma: case report and literature review.

Abstract

To describe a unique case of scleroderma (SSc) presenting as multiple keloidal nodules and early-onset osteoarthritis (OA), and to summarize the clinical and serological data for 13 similar patients reported in the English literature since 1966. MEDLINE review of the literature over a 35-year period (1966-2002) revealed 13 cases of nodular SSc. We describe a case of nodular SSc in a 40-year-old African-American male with localized SSc who developed progressive skin thickening and keloidal nodules on the arms, hands, chest, abdomen, and thighs with advanced osteoarthritis of the hips. In all 14 cases, diagnosis was made based on skin biopsy and evidence of keloid (nodule) formation. Ten cases occurred in women and 4 in men, with ages ranging from 9 to 66 years and a mean age of 38.9 years. The ethnicity of the patients was given in only 5 of the 13 previously reported cases. Including our patient, 4 were of African descent, and 2 were Caucasian. Most patients had symptoms of SSc consisting of arthralgias (n = 10), sclerodactyly (n = 9), Raynaud's phenomenon (n = 8), digital pitting and/or calcinosis (n = 5), shortness of breath with pulmonary fibrosis (n = 5) or pulmonary hypertension (n = 1), dysphagia or reflux (n = 3), renal disease (n =3), and elevated erythrocyte sedimentation rate (n = 3). Nodular SSc is a rare variant that presents with lesions that clinically resemble keloids. OA, as documented in the present case, does not appear to be a typical feature of nodular SSc.