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A Review of 245 Adult and Pediatric Cases

Justin J. Leitenberger, BS; Rachael L. Cayce, BS; Robert W. Haley, MD; Beverley Adams-Huet, MS; Paul R. Bergstresser, MD; Heidi T. Jacobe, MD

Arch Dermatol. 2009;145(5):545-550.

Objective  To determine the prevalence of extracutaneous manifestations and autoimmunity in adult and pediatric patients with morphea.

Design  A retrospective review of 245 patients with morphea.

Setting  University of Texas Southwestern Medical Center–affiliated institutions.

Patients  Patients with clinical findings consistent with morphea.

Main Outcome Measures  Prevalence of concomitant autoimmune diseases, prevalence of familial autoimmune disease, prevalence of extracutaneous manifestations, and laboratory evidence of autoimmunity (antinuclear antibody positivity). Secondary outcome measures included demographic features.

Results  In this group, adults and children were affected nearly equally, and African Americans were affected less frequently than expected. The prevalence of concomitant autoimmunity in the generalized subtype of morphea was statistically significantly greater than that found in all other subtypes combined (P = .01). Frequency of a family history of autoimmune disease showed a trend in favor of generalized and mixed subgroups. The linear subtype showed a significant association with neurologic manifestations, while general systemic manifestations were most common in the generalized subtype. Antinuclear antibody positivity was most frequent in mixed and generalized subtypes.

Conclusions  High prevalences of concomitant and familial autoimmune disease, systemic manifestations, and antinuclear antibody positivity in the generalized and possibly mixed subtypes suggest that these are systemic autoimmune syndromes and not skin-only phenomena. This has implications for the management and treatment of patients with morphea.

Author Affiliations: Departments of Dermatology (Mr Leitenberger, Ms Cayce, and Drs Bergstresser and Jacobe), Internal Medicine (Dr Haley and Ms Adams-Huet), and Clinical Sciences (Drs Haley and Bergstresser and Ms Adams-Huet), The University of Texas Southwestern Medical Center at Dallas.

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