Distinct autoimmune syndromes in morphea

A review of 245 adult and pediatric cases

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

Research output: Contribution to journalArticle

140 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)545-550
Number of pages6
JournalArchives of Dermatology
Volume145
Issue number5
DOIs
StatePublished - May 2009

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Localized Scleroderma
Pediatrics
Autoimmune Diseases
Antinuclear Antibodies
Autoimmunity
Outcome Assessment (Health Care)
Neurologic Manifestations
African Americans
Demography
Skin

ASJC Scopus subject areas

  • Dermatology

Cite this

Distinct autoimmune syndromes in morphea : A review of 245 adult and pediatric cases. / Leitenberger, Justin J.; Cayce, Rachael L.; Haley, Robert W.; Adams-Huet, Beverley; Bergstresser, Paul R.; Jacobe, Heidi T.

In: Archives of Dermatology, Vol. 145, No. 5, 05.2009, p. 545-550.

Research output: Contribution to journalArticle

Leitenberger, Justin J. ; Cayce, Rachael L. ; Haley, Robert W. ; Adams-Huet, Beverley ; Bergstresser, Paul R. ; Jacobe, Heidi T. / Distinct autoimmune syndromes in morphea : A review of 245 adult and pediatric cases. In: Archives of Dermatology. 2009 ; Vol. 145, No. 5. pp. 545-550.
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