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J Eur Acad Dermatol Venereol. 2010 Sep;24(9):1031-4. doi: 10.1111/j.1468-3083.2010.03572.x. Epub 2010 Feb 25.

The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study.

Author information

1
Department of Dermatology, Churchill Hospital, Oxford, UK. victoria.j.sherman@doctors.org.uk

Abstract

BACKGROUND:

Familial lichen sclerosus (LS) has been described in only 37 families. We feel that the association is under-reported.

OBJECTIVES:

To determine the percentage of patients with LS who have a positive family history.

METHOD:

A large observational-cohort study of a total of 1052 females at vulval clinics within a University Hospital with a diagnosis of LS of the vulva (clinical diagnosis was confirmed in 80% of cases by histology). Patients were questioned as to family history of LS or balanitis xerotica obliterans; male circumcision for medical reasons; vulval cancer; and routine medical and family history. The outcome was the presence or absence of personal or family history of LS, autoimmune disorder or vulval cancer.

RESULTS:

In total 1052 patients were investigated. Of these, 126 (12%) had a positive family history of LS. These patients belonged to 95 families. Vulval cancer was significantly increased in those with a family history of LS compared with those without (4.1% vs. 1.2%, P < 0.05). There was more associated autoimmune disease in familial LS than in sporadic LS, although this was not statistically significant. (7% vs. 5%, P > 0.2).

CONCLUSION:

Our data from a large cohort of patients with LS provide evidence of an increased risk for family members to develop LS. This indicates a likely genetic component in the aetiology of LS.

[Indexed for MEDLINE]

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