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ORIGINAL ARTICLE

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

V Sherman

Corresponding Author

Department of Dermatology, Churchill Hospital, Oxford, UK

V Sherman. E‐mail:

victoria.j.sherman@doctors.org.uk

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T McPherson

Department of Dermatology, Churchill Hospital, Oxford, UK

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M Baldo

Department of Dermatology, Churchill Hospital, Oxford, UK

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A Salim

Department of Dermatology, Birmingham Heartlands Hospital, Birmingham, UK

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XH Gao

Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China

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F Wojnarowska

Department of Dermatology, Churchill Hospital, Oxford, UK

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First published: 02 August 2010
Cited by: 9

Conflict of interest
No conflict of interests.

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.

Number of times cited: 9

  • , Oral lichen sclerosus: a systematic review of reported cases and two new cases, International Journal of Dermatology, 57, 5, (521-528), (2018).
  • , The treatment of vulval lichen sclerosus in prepubertal girls: a critically appraised topic, British Journal of Dermatology, 176, 2, (307-316), (2017).
  • , New insights into potential risk factors and associations in genital lichen sclerosus: Data from a multicentre Italian study on 729 consecutive cases, Journal of the European Academy of Dermatology and Venereology, 31, 4, (699-704), (2016).
  • , Dermatoses of the Female Genitalia, Rook's Textbook of Dermatology, Ninth Edition, (1-52), (2016).
  • , Pediatric Lichen Sclerosus: A Review of the Epidemiology and Treatment Options, Pediatric Dermatology, 32, 5, (593-599), (2015).
  • , Evidence‐based (S3) Guideline on (anogenital) Lichen sclerosus, Journal of the European Academy of Dermatology and Venereology, 29, 10, (e1-e43), (2015).
  • , Vulval Lichen sclerosus, Erosive Lichen Planus, and Vulvodynia, Evidence‐Based Dermatology, (615-623), (2014).
  • , Clinical Scoring System for Vulvar Lichen Sclerosus, The Journal of Sexual Medicine, 9, 9, (2342-2350), (2012).
  • , Balanitis xerotica obliterans: a review of diagnosis and management, International Journal of Dermatology, , (2018).