Childhood lichen sclerosus is a rare but important diagnosis

Dan Med J. 2012 May;59(5):A4424.

Abstract

Introduction: Lichen sclerosus (LS) is a chronic skin disorder with a predilection for the anogenital area. The disease is mostly seen in prepubertal and postmenopausal females. The lesions present as sharply demarcated white plaques encircling the vagina and anus. The atrophic form can lead to scarring of the affected area.

Material and methods: Retrospective analysis of hospital records of children (aged 1-18 years) seen at the Department of Dermatology and Allergy Centre in Odense from October 1998 to November 2010 with a definite clinical diagnosis of anogenital LS with/without a confirming biopsy.

Results: A total of 35 girls and one boy were diagnosed with anogenital LS. The diagnostic delay was 17 months. Pruritus, dysuria, bleeding and constipation were the dominant complaints, while one patient was asymptomatic. Referral was made by general practitioners, private dermatologists and paediatricians. Sexual abuse was suspected in five cases. Ten patients underwent biopsy confirming LS. Before a definite diagnosis was given, many children were extensively treated with various topical and oral agents. In our outpatient clinic, 30 children were treated with potent/ultra-potent corticosteroids and five patients were treated with calcineurin inhibitors.

Conclusion: General practitioners may overlook this disorder despite characteristic clinical features and effective symptomatic treatment. Diagnostic delay is a significant problem for both patient and family, and the lesions may mimic the findings of sexual abuse. Potent corticosteroids are very effective in symptomatic treatment.

Funding: not relevant.

Trial registration: not relevant.

Publication types

  • Evaluation Study

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Child
  • Child, Preschool
  • Delayed Diagnosis
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Lichen Sclerosus et Atrophicus / diagnosis*
  • Lichen Sclerosus et Atrophicus / drug therapy
  • Male
  • Retrospective Studies
  • Tacrolimus / analogs & derivatives
  • Tacrolimus / therapeutic use
  • Treatment Outcome
  • Vulvar Lichen Sclerosus / diagnosis*
  • Vulvar Lichen Sclerosus / drug therapy

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • pimecrolimus
  • Tacrolimus