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

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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.

Section snippets

Patients

A retrospective chart review of patients with LoS who presented at the outpatient clinic for connective tissue diseases of the Department of Dermatology at Ruhr University Bochum was performed. Observation period was 11 years (January 1, 2000, through December 31, 2010). A protocol on the evaluation of connective tissue diseases including LoS and LiS (individual ethics submission code: 2851) was approved by the ethics review board of the Ruhr University Bochum.

Clinical examination and classification of LoS subtypes

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Demographic characteristics

A total of 472 patients with LoS were given a diagnosis and treated at the Department of Dermatology at Ruhr University Bochum from January 1, 2000, through December 31, 2010. The demographic characteristics are detailed in Table I. Similar to previous reports, there was a much higher proportion of women affected by the disease, with an overall female to male ratio of 3.5:1 in adults and 8:1 in children.

Distribution of LoS subtypes

The distribution of LoS subtypes in the overall cohort, and in adults and children is

Discussion

This retrospective analysis of a large cohort of patients with LoS demonstrates that an essential proportion of these patients have concomitant LiS. The demographics and subtype distribution of our cohort largely confirms previous investigations, showing that the majority of patients with LoS are women, that the plaque type (morphea) is most frequent in adults, and that linear types of LoS dominate in childhood.2, 3, 4

The possible coexistence of both LoS and LiS has been well known for many

Conclusions

Accumulating evidence exists that a subset of patients with LoS, namely those with morphea and generalized LoS, have a significantly increased risk for the development of concomitant LiS. This high LiS prevalence in LoS is suggestive for a common pathogenetic background. Moreover, patients with LoS frequently present other autoimmune disorders. These findings should definitively have an impact on our practical management. Clinical examination of patients with LoS should include inspection of

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Funding sources: None.

Conflicts of interest: None declared.

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