Adult Langerhans cell histiocytosis limited to the skin

Dermatology. 2003;207(2):157-61. doi: 10.1159/000071786.

Abstract

Background: The incidence of Langerhans cell histiocytosis (LCH) is 4-5 per million in children with only 30% of this number having an adult onset. While dermatological manifestations occur in as many as 50% of cases, disease limited to the skin is uncommon among reported cases of adult LCH.

Objectives and methods: To present 3 new cases of adult LCH and a review of the literature of isolated cutaneous LCH in adults.

Results: Three adults with scalp, vulvar and generalized LCH lesions had refractory responses to treatment.

Conclusions: LCH may present with unusual cutaneous manifestations limited to the skin in adults. Optimal treatment has not yet been determined.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Female
  • Histiocytosis, Langerhans-Cell / pathology*
  • Humans
  • Male
  • Middle Aged
  • Scalp / pathology
  • Skin / pathology
  • Skin Diseases / pathology*
  • Vulva / pathology