Conflict of interest No conflict of interests.
The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study
Article first published online: 25 FEB 2010
© 2010 The Authors. Journal compilation © 2010 European Academy of Dermatology and Venereology
Journal of the European Academy of Dermatology and Venereology
Volume 24, Issue 9, pages 1031–1034, September 2010
How to Cite
Sherman, V., McPherson, T., Baldo, M., Salim, A., Gao, X. and Wojnarowska, F. (2010), The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study. Journal of the European Academy of Dermatology and Venereology, 24: 1031–1034. doi: 10.1111/j.1468-3083.2010.03572.x
- Issue published online: 2 AUG 2010
- Article first published online: 25 FEB 2010
- Received: 18 August 2009; Accepted: 19 December 2009
- lichen sclerosus;
- vulval cancer
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.