Isomorphic and symmetric adult-onset generalized morphea are associated with distinctive clinical features: A retrospective multicenter study

To the Editor: Generalized morphea (GM) is a rare subtype of localized scleroderma characterized by widespread involvement. GM defined presence at least 4 lesions larger than 3 cm that involve 2 anatomic sites.1Laxer R.M. Zulian F. Localized scleroderma.Curr Opin Rheumatol. 2006; 18: 606-613Crossref PubMed Scopus (243) Google Scholar Isomorphic and symmetric subsets were recently delineated based on computerized lesion mapping approach.2Teske N. Welser J. Jacobe H. Skin for classification generalized morphea.J Am Acad Dermatol. 2018; 78: 351-357Abstract Full Text PDF (6) In isomorphic pattern, favor areas skin friction (ie, brassiere band, waistband, inguinal creases), whereas in one, they occur with distribution trunk extremities. A higher frequency deep involvement was reported patients GM, lichen sclerosus-like changes frequent finding plaques from GM.2Teske This retrospective study aimed to describe clinical characteristics another cohort adult-onset investigate if further differences exist between subsets. We conducted chart review 27 followed up hospitals east France 2009 2019. Cases identified using international coding (L94.0 International Classification Diseases, 10th revision). Data including demographics, comorbidities, laboratory findings retrieved. Two investigators (AB CL) independently reviewed photographs categorize into or subsets, when possible, published.2Teske third blinded investigator (DL) involved case disagreement. Comparison made Student t test Fisher's exact test. Interrater agreement assessed Cohen kappa The relevant demographic patient comorbidities are summarized Table I. All had undergone systematic genital examination. Fifteen (56%) sclerosus, 9 (33%) autoimmune comorbidities. Twenty-three (85%) could be classified either (n = 12) 11) remaining unclassed features both none. Cohen's 0.88, suggesting very good raters applicability this classification.Table IDemographic associated GMCharacteristicsValuePatients, N27Sex Female, n (%)21 (78) Male, (%)6 (22) F:M3.5:1Age onset, y, mean (SD)60 (11)Time diagnosis, mo, (SD)18 (26)Genital (%)15 (56)Autoimmune (%)9 (33) Autoimmune thyroiditis2 Inflammatory bowel disease1 Alopecia areata1 Systemic lupus erythematosus1 Spondyloarthropathy1 Psoriasis1 Rheumatoid arthritis1 Polymyalgia rheumatica1F, Female; M, male; SD, standard deviation. Open table new tab F, comparative biological II. Sex age onset equivalent groups. Genital sclerosus strikingly more subset (10/12 vs 4/11; P .04), often (1/12 6/11; .03).Table IIComparison GMCharacteristicsIsomorphic GMSymmetric GMP∗P < .05 considered significant.Patients, (%)†Four did not fit subset.12 (52)11 (48)Sex, (%) Female10 (83)8 (73).64 Male2 (17)3 (27)Age (SD)63 (12)57 (10).20Pruritus, (%)4 (33)7 (64).22Functional impairment, (%)0 (0)3 (27).09Genital (%)10 (83)4 (36).04Autoimmune (%)1 (8)6 (54.5).03Antinuclear antibodies positivity, n/total (%)‡≥1/160; only 17 tested (7 isomorphic, 11 symmetric).1/7 (14)4/10 (40).34GM, morphea; Bold text signifies statistical significance (<.05).∗ significant.† Four subset.‡ ≥1/160; symmetric). (<.05). found an even prevalence already classical morphea.3Lutz V. Francès C. Bessis D. et al.High prospective series 76 morphea: toward better understanding spectrum morphea.Arch 2012; 148: 24-28Crossref (32) peaked subgroup Despite its design small sample, our strongly supports relevance dichotomous classification. Taken together, published Teske al2Teske results suggest whether location not, may represent most superficial manifestation morphea, where subtle repetitive trauma would play determinant pathophysiologic role.