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Distinct Autoimmune Syndromes in Morphea a Review of 245 Adult and Pediatric Cases

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Distinct Autoimmune Syndromes in Morphea a Review of 245 Adult and Pediatric Cases STUDY Distinct Autoimmune Syndromes in Morphea 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 Objective: To determine the prevalence of extracuta- fected less frequently than expected. The prevalence of neous manifestations and autoimmunity in adult and pe- concomitant autoimmunity in the generalized subtype diatric patients with morphea. of morphea was statistically significantly greater than that found in all other subtypes combined (P=.01). Fre- Design: A retrospective review of 245 patients with quency of a family history of autoimmune disease showed morphea. a trend in favor of generalized and mixed subgroups. The linear subtype showed a significant association with neu- Setting: University of Texas Southwestern Medical Cen- rologic manifestations, while general systemic manifes- ter–affiliated institutions. tations were most common in the generalized subtype. Antinuclear antibody positivity was most frequent in Patients: Patients with clinical findings consistent with mixed and generalized subtypes. morphea. Conclusions: High prevalences of concomitant and fa- Main Outcome Measures: Prevalence of concomi- tant autoimmune diseases, prevalence of familial auto- milial autoimmune disease, systemic manifestations, and immune disease, prevalence of extracutaneous manifes- antinuclear antibody positivity in the generalized and pos- tations, and laboratory evidence of autoimmunity sibly mixed subtypes suggest that these are systemic au- (antinuclear antibody positivity). Secondary outcome toimmune syndromes and not skin-only phenomena. This measures included demographic features. has implications for the management and treatment of patients with morphea. Results: In this group, adults and children were af- fected nearly equally, and African Americans were af- Arch Dermatol. 2009;145(5):545-550 ORPHEA IS CHARACTER- than-expected prevalence of familial auto- ized by sclerosis of the immune disease.6,7 The same pediatric study skin and, in some found systemic manifestations outside the cases, underlying tis- areaofmorpheain22.4%ofchildren,includ- sue. It is generally ing arthralgias and esophageal dysmotility. Mthought to be an autoimmune disorder af- Datainadultsarelimited,with1case-control fecting a single organ—the skin. Conse- study5 of 50 adult white women reporting in- quently, current classification schemes di- creased personal and familial autoimmunity, vide morphea into categories based solely but antinuclear antibody (ANA) status and on cutaneous morphology, without refer- systemic manifestations were not reported. ence to systemic disease or autoimmune Many autoantibodies have been reported in Author Affiliations: phenomena. This classification is likely in- patientswithmorphea,includingANA,anti– Departments of Dermatology (Mr Leitenberger, Ms Cayce, complete. single-stranded DNA, antihistone, anti– and Drs Bergstresser and Rather, morphea may be a systemic au- topoisomerase II␣, antiphospholipid, and Jacobe), Internal Medicine toimmune condition.1-8 For example, case rheumatoid factor.9-12 The clinical and prog- (Dr Haley and reports describe morphea coexisting with nostic significance of these autoantibodies Ms Adams-Huet), and Clinical other autoimmune diseases, including sys- remains unclear. Sciences (Drs Haley and temic lupus erythematosus, vitiligo, pri- Taken together, these reports suggest that Bergstresser and Ms Adams-Huet), mary biliary cirrhosis, autoimmune hepa- morphea is an autoimmune disease with a The University of Texas titis, Hashimoto thyroiditis, and myasthenia spectrum of manifestations ranging from 1-5,8,9 Southwestern Medical Center gravis. A retrospective review of 750 only skin to multiple organ involvement. at Dallas. children with morphea revealed a greater- What remains unclear is the prevalence of (REPRINTED) ARCH DERMATOL/ VOL 145 (NO. 5), MAY 2009 WWW.ARCHDERMATOL.COM 545 ©2009 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 ter review of the medical records, all patients who were con- Table 1. Main Demographic Characteristics of 245 Patients sidered to have met the clinical criteria for morphea With Morphea (histopathologic results were reviewed when present but were not necessary for inclusion) were included for analysis. Pa- Characteristic Valuea tients who were found on review of the medical record to have Age at onset, y, mean (SD) lichen sclerosus alone or sclerosing skin conditions other than Adults 44.1 (17.9) morphea were excluded (186 of 431). Children 8.7 (3.3) For the 245 patients who met the inclusion criteria, data were Sex, No. entered electronically into an Access database (Microsoft, Red- Overall mond, Washington). Data extraction included demographic in- Female 199 formation, clinical variables, histologic data, laboratory data, Male 46 medical history of rheumatic or other autoimmune disease, fam- F/M ratio 4:1 ily history of rheumatic or other autoimmune diseases in first- Adults 123 and second-degree relatives, and review of systems. Female 103 Morphea subtypes were determined from the medical rec- Male 20 ord when specified or from the physical examination findings F/M ratio 5:1 when not specified in the record. The subtypes investigated were Children 122 plaque, linear (including linear scleroderma, en coup de sa- Female 96 bre, and progressive hemifacial atrophy), generalized, mixed Male 26 (defined by the presence of 2 subtypes in the same patient; how- F/M ratio 3:1 ever, if 1 subtype was generalized, patients were categorized Race/ethnicity, No. (%) as generalized), lichen sclerosus/morphea overlap, guttate, bul- Overall 245 lous, and deep, using established clinical criteria; none of the White 178 (72.7) 14,15 Hispanic/Latino 34 (13.9) patients had bullous morphea. Asian 6 (2.4) Summary statistics were used for reporting demographic, African American 11 (4.5) clinical, and laboratory characteristics. The Fisher exact test Other 16 (6.5) was used to test the association between the 5 subtypes of mor- Adults 123 phea and the presence or absence of autoimmune disease, fam- White 93 (71.5) ily history of autoimmune disease, and systemic findings. Be- Hispanic/Latino 10 (7.7) cause ANAs were tested in relatively few patients, results are Asian 3 (2.3) reported as frequency counts. PϽ.05 was considered statisti- African American 7 (5.4) cally significant. SAS software version 9.1.3 (SAS Institute, Cary, Other 10 (7.7) North Carolina) was used for data analysis. Unavailable 7 (5.4) Children 122 White 85 (69.7) RESULTS Hispanic/Latino 24 (19.7) Asian 3 (2.5) DEMOGRAPHICS African American 4 (3.3) Other 6 (4.9) Table 1 shows the demographic characteristics for the Abbreviations: F, female; M, male. patients. Similar to prior reports, there were 199 fe- a Percentages do not total 100 because of rounding. males (81.2%) and 46 males (18.8%), with an overall fe- male to male ratio of 4.2:1. The racial distribution in all of the patients with mor- autoimmune and systemic disease in morphea and its sub- phea was 72.7% white, 13.9% Hispanic, 4.5% African types and how these findings correlate with the autoanti- American, 2.4% Asian, and 6.5% other (Pacific Islander, body profile. This uncertainty negatively affects patient care Native American, etc). Of the 245 patients, 123 (50.2%) because many patients are untreated or are treated only with had onset of morphea as an adult and 122 (49.8%) de- skin-directed therapy and may require systemic therapy. veloped morphea as a child. The mean age of onset was This study aims to address this gap in knowledge by quan- 8.7 years for children compared with 44.1 years for adults. tifying the prevalence of autoimmunity and systemic mani- festations in adults and children with morphea. SUBTYPE DISTRIBUTION METHODS Table 2 shows subtype distribution among the 245 pa- tients with morphea. The most common subtype over- Approval was obtained from the institutional review board to all was plaque at 35.9% (n=88) and then linear at 25.7% review the medical records of patients with morphea seen from (n=63). Deep and guttate subtypes were seen in less than June 1, 2001, to June 1, 2007, at University of Texas South- 1.2% (3 each) of all patients and are not included in sub- western Medical Center at Dallas–affiliated institutions (Uni- sequent analyses. Subtype distribution differed between versity of Texas Southwestern Medical Center at Dallas uni- adults and children. versity-based faculty practice, Texas Scottish Rite Hospital, Children’s Medical Center in Dallas, and Parkland Memorial Hospital). A total of 431 potential adult and pediatric patients Adults were identified on the basis of the International Classification of Diseases, Ninth Revision diagnosis code for morphea/ The most common subtypes were plaque-type morphea localized scleroderma and lichen sclerosus (code 701.0).13 Af- in 43.9% (54 of 123) of adult patients and generalized (REPRINTED) ARCH DERMATOL/ VOL 145 (NO. 5), MAY 2009 WWW.ARCHDERMATOL.COM 546 ©2009 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 Table 2. Subtype Distribution in 245 Patients With Morpheaa Morphea Subtype, No. (%) of Patients Group Overall Plaque Linear Generalized Deep Guttate Lichen Sclerosus/Morphea Mixed Overall 245 88 (35.9) 63 (25.7) 37 (15.1) 3 (1.2) 3 (1.2) 18 (7.3) 33 (13.5) Adults 123 54 (43.9) 12 (9.8) 29 (23.6) 2 (1.6) 3 (2.4) 18 (14.6) 5 (4.1) Children 122 34 (27.9) 51 (41.8) 8 (6.6) 1 (0.8) 0 0 28 (23.0) a Percentages do not total 100 because of rounding. in 23.6% (29 of 123) of adult patients. The mixed sub- type was seen in 4.1% (5 of 123) of adult patients. Table 3. Rheumatic Disease and Other Autoimmune Disorders in Patients With Morphea and Their Relatives Children No. (%) of Patients The most common subtype was linear in 41.8% (51 of Concomitant Familial 122) of pediatric patients, followed by plaque and mixed Disorder Disorder subtypes at 27.9% (34 of 122) and 23.0% (28 of 122), Overall (N=245) 43 (17.6) 40 (16.3) respectively.
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