Widespread skin-limited adult Langerhans cell histiocytosis: long-term follow-up with good response to interferon alpha

Langerhans cell histiocytosis (LCH) most frequently involves bone, but also involves the skin in 40% of cases; in 10% of patients it is limited to the skin. Cutaneous findings of skin-limited LCH are scaly papules, vesicles, nodules, tumours with erosion, ulceration, or crusting and/or purpura. We report a case of widespread adult-onset LCH confined to skin in which topical carmustine, photochemotherapy, systemic steroids, and 2-chlorodeoxyadenosine were only partially effective. However, longer remission was achieved by the use of subcutaneous interferon-alpha2b.