Adult-onset Langerhans cell histiocytosis confined to the skin

Date

2006

Authors

Journal Title

Journal ISSN

Volume Title

Publisher

Wiley

Abstract

Langerhans cell histiocytosis (LCH) is a clonal, pleo-morphic disease of unknown aetiology, with theaccumulation of local or disseminated atypical histiocyticcells staining positively for S-100 and CD-1a, and causingdamage in the bones, lungs, mucocutaneous structuresand endocrine organs.1 The condition is generally dia-gnosed in infancy and childhood, but onset in adulthoodcan occasionally occur. The eruption is usually diffuse ormanifests as part of a multisystemic disease, and up to 25–50% of patients with LCH will present initially with acutaneous rash.1–5 Cutaneous manifestations are hetero-genous and similar both in children and adults.1,2 LCHconfined to the skin is uncommon among reported casesof adults.2–8 We describe a rare case of adult-onset LCHpresenting as cutaneous lesions located symmetrically onthe scalp, groin and inframammarian areas, and review theliterature for previous adult cases of isolated cutaneousLCH.

Description

Keywords

Dermatology, Cutivate, Deltacortril

Citation

Aydoğan, K. vd. (2006). ''Adult-onset Langerhans cell histiocytosis confined to the skin''. Journal of the European Academy of Dermatology and Venereology, 20(7), 890-892.