Adult Langerhans cell histiocytosis limited to the skin

Asha Singh; Victor G. Prieto; Adam Czelusta; Kenneth L. McClain; Madeleine Duvic

doi:10.1159/000071786

Adult Langerhans cell histiocytosis limited to the skin

Asha Singh, Victor G. Prieto, Adam Czelusta, Kenneth L. McClain, Madeleine Duvic

Research output: Contribution to journal › Article › peer-review

50 Scopus citations

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.

Original language	English (US)
Pages (from-to)	157-161
Number of pages	5
Journal	Dermatology
Volume	207
Issue number	2
DOIs	https://doi.org/10.1159/000071786
State	Published - 2003

Keywords

Histiocytosis X
Labiectomy
Langerhans cell histiocytosis
N,N-diethyl-m-toluamide
Staphylococcus aureus
Superantigen

ASJC Scopus subject areas

Dermatology

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10.1159/000071786

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title = "Adult Langerhans cell histiocytosis limited to the skin",

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.",

keywords = "Histiocytosis X, Labiectomy, Langerhans cell histiocytosis, N,N-diethyl-m-toluamide, Staphylococcus aureus, Superantigen",

author = "Asha Singh and Prieto, {Victor G.} and Adam Czelusta and McClain, {Kenneth L.} and Madeleine Duvic",

year = "2003",

doi = "10.1159/000071786",

language = "English (US)",

volume = "207",

pages = "157--161",

journal = "Dermatology",

issn = "1018-8665",

publisher = "S. Karger AG",

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TY - JOUR

T1 - Adult Langerhans cell histiocytosis limited to the skin

AU - Singh, Asha

AU - Prieto, Victor G.

AU - Czelusta, Adam

AU - McClain, Kenneth L.

AU - Duvic, Madeleine

PY - 2003

Y1 - 2003

N2 - 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.

AB - 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.

KW - Histiocytosis X

KW - Labiectomy

KW - Langerhans cell histiocytosis

KW - N,N-diethyl-m-toluamide

KW - Staphylococcus aureus

KW - Superantigen

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Adult Langerhans cell histiocytosis limited to the skin

Abstract

Keywords

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