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Rare disease caused by an unusual large number of Langerhans cells accumulating

Hand-Schuller-Christian disease (also known as chronic disseminated histiocytosis X) is a rare form of histiocytosis X, in which large nests of monoclonal Langerhans Cells (also known as histiocytes) are found in various tissues and cause to damage them. These cells are dendritic cells normally sitting in your skin as your first line of defense against unwanted invaders, but noone knows why some children present with them everywhere. A genetic or autoimmune reason seems the most logical.

First described by Alfred Hand, Arthur Schuller and Henry Asbury Christian this disease commonly presents in children between 3 to 6 with diabetes insipidus (the unability to retain water, meaning that patients pee and drink a lot), exophthalmus (bulging eyes), chronic otitis media , short stature and seborrhoeic dermatitis of the scalp. Additionally to that they might have a large number of purple spots in their skin and swollen lymph glands.

Xrays might show numerous bony lesions with a punched out appearance and lesions in the patient's lungs. A full blood count often shows signs of anaemia, leukopenia (lack of white blood cells) and thrombocytopenia (lack of platelets). To fully diagnose the disease, a skin or bone biopsy has to be taken and examined by the histopathologists for the presence of large numbers of histiocytes.

Prognosis depends on the time of diagnosis and the distribution of the lesions. In children, a good response has been shown to a mixture of steroids and chemotherapy (i.e. vincristin. 35% of all patients completely recover, 15% die.

Source:
Vaze AM, Koppikar MG, Patel MS, Phadke PP, Nadkarni MM, Bapat RD. Hand Schuller Christian disease. J Postgrad Med serial online 1980 cited 2004 Apr 13 ;26:138-41
Oxford Handbook of Clinical Medicine
http://www.umm.edu/ency/article/000068.htm

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