Unusual manifestation of Langerhans' cell histiocytosis

An unusual manifestation of the disease Langerhans' cell histiocytosis (LHC) is presented. An 18-year-old man was admitted to the Department of Dermatology after he had been suffering for some time from affections of the skin in the scalp and perianal region which were resistant to treatment. Shortly after LHC was diagnosed, he developed spontaneous bilateral pneumothorax. In his past life he had been without cardiopulmonary complaints. In addition to this, he developed a diabetes insipidus, originating in connexion with the present disease. The combination of skin affection in the perianal region, spontaneous bilateral pneumothorax and diabetes insipidus in relation to LHC has not been described previously.