Adult-onset Langerhans cell histiocytosis confined to the skin

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.