Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases.
OBJECTIVE
To determine the prevalence of extracutaneous manifestations and autoimmunity in adult and pediatric patients with morphea.


METHODS
A retrospective review of 245 patients with morphea.


METHODS
University of Texas Southwestern Medical Center-affiliated institutions. Patients Patients with clinical findings consistent with morphea.


METHODS
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.
Leitenberger
Justin J
JJ
Department of Dermatology, UT Southwestern Medical Center, Dallas, TX 75390-9069, USA.
Cayce
Rachael L
RL
Haley
Robert W
RW
Adams-Huet
Beverley
B
Bergstresser
Paul R
PR
Jacobe
Heidi T
HT
D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology* / Q000276:immunology; D015551:Autoimmunity / Q000276:immunology*; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D013577:Syndrome; D013781:Texas / Q000453:epidemiology; D013997:Time Factors
D000974:Antibodies, Antinuclear