LAUSR

2-CdA: 2-chlorodeoxyadenosine XD: xanthoma disseminatum INTRODUCTION Xanthoma disseminatum (XD) is a rare, noneLangerhans cell histiocytosis characterized by a clinical triad of cutaneous xanthomas, xanthomas of mucous membranes, and diabetes insipidus. Laboratory values, including serum lipid levels, are usually normal. Mucous membrane involvement is seen in 40% to 60% of patients, most commonly in the oropharynx and larynx. Although benign, extensive skin lesions can be unsightly, and systemic involvement can lead to hoarseness, dysphagia, and diabetes insipidus and can result in increased mortality when critical organs are involved. Attempted treatment modalities range widely and with varied success, using cyclophosphamide, lipid-lowering agents, CO2 laser 5 and 2-chlorodeoxyadenosine (2CdA) also known as cladribine. Here we report a case of XD presenting with skin and symptomatic laryngopharyngeal lesions, with striking resolution after treatment with 2-CdA.