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J Rheumatol. 2003 Nov;30(11):2500-2.

Nodular scleroderma: case report and literature review.

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  • 1Department of Internal Medicine, Medical University of South Carolina, 96 Jonathan Lucas Street, Charleston, SC 29425, USA.

Abstract

OBJECTIVE:

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.

METHODS:

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.

RESULTS:

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).

CONCLUSION:

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.

PMID:
14677198
[PubMed - indexed for MEDLINE]
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