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Clinical Problem Solving: Radiology
February 1, 2008

Radiology Quiz Case 1: Diagnosis

Author Affiliations

Patricia A.HudginsMD

Arch Otolaryngol Head Neck Surg. 2008;134(2):212. doi:10.1001/archoto.2007.31-b

The patient was transferred to a skull base unit, where the findings of another biopsy led to the diagnosis of LCH. A whole-body bone scan showed that the disease was limited to the left temporal bone, and radiotherapy to the left side of skull was initiated.

In 1953, Lichtenstein1observed that eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease all have similar histologic features and coined the term histiocytosis X. In 1987, the name was changed to LCH, as recommended by the Writing Group of the Histiocytosis Society.2Langerhans cell histiocytosis is a rare, proliferative disorder in which there is accumulation of pathologic Langerhans cells, causing local tissue infiltration and destruction.3Histiocytes, which include dendritic cells and mononuclear phagocytes, are derived from bone marrow and migrate into the peripheral tissues after a period in the circulation. Langerhans cells are a subpopulation of dendritic histiocytes and are primarily located in the basal layer of the epidermis. Langerhans cell histiocytosis is rare in adults and principally affects children younger than 4 years.3It has an incidence of 0.5 to 5 cases per 1 million children per year.3Boys are affected twice as often as girls.3