Coexistence of lichen sclerosus and morphea: a retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center

Abstract

Background: The coexistence of lichen sclerosus (LiS) and localized scleroderma (LoS) has sporadically been reported in the literature. Recently, a prospective multicenter study demonstrated a surprisingly high percentage of genital LiS in patients with morphea.

Objective: The aim of this study was to determine the prevalence of LiS in a cohort of patients with LoS who presented at a tertiary referral medical center for connective tissue diseases in Germany.

Methods: We retrospectively evaluated the prevalence of genital and extragenital LiS in adult and pediatric patients with different subtypes of LoS. Secondary outcome measures included demographic characteristics and prevalence of other concomitant autoimmune diseases.

Results: Of the 472 patients (381 adults, 91 children; mean age: 46 years; range, 4-88 years; female to male ratio: 3.5:1 in adults and 8:1 in children) with LoS, 27 (5.7%) also presented with LiS (19 extragenital and 8 genital lesions). LiS exclusively occurred in patients with plaque-type (morphea) and generalized LoS. Twenty-six of the 27 (96.2%) patients with concomitant LoS and LiS were adults. Compared with LiS in the general population, LiS was significantly more frequent in LoS as indicated by an odds ratio of 18.1 (95% confidence interval 2.6-134.2; P < .0001). In all, 38 (8.1%) patients with LoS had other autoimmune disorders (most frequently Hashimoto thyroiditis, rheumatoid arthritis, and alopecia areata).

Limitations: This was a retrospective study.

Conclusions: This large retrospective analysis confirms recent reports of a high prevalence of LiS in patients with LoS. Based on these findings, patients with LoS, especially those with morphea, should be carefully screened for concomitant LiS, including inspection of the anogenital region.