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Clinical Problem Solving: Pathology
January 2005

Pathology Quiz Case—Diagnosis

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Copyright 2005 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2005

Arch Otolaryngol Head Neck Surg. 2005;131(1):82. doi:10.1001/archotol.131.1.82

Juvenile xanthogranuloma was first described in 1905 by Adamson,1 who noted multiple yellow cutaneous papules that eventually regressed spontaneously in a 2-year-old boy. It is a benign histiocytic lesion that most often involves the head and neck region, followed by the trunk, upper extremities, and lower extremities, respectively.2 It usually presents as single or multiple yellow-orange to red-brown cutaneous lesions ranging in size from a few millimeters to several centimeters. Numerous extracutaneous sites within the head and neck region have been described, including the eye,3 oral cavity,4 temporal bone,5 nasopharynx,6 and larynx.7 The nasal cavity is an uncommon site of presentation, with few descriptions available in the English-language literature.4,8,9 Males are preferentially affected in the juvenile form (male-female ratio, 4:1), although a similar predilection does not exist in the adult form.2 The lesion is 10 times more common in whites than in blacks. Despite the nomenclature, JXG has been found to arise in both pediatric and adult populations, with presentations most often occurring in the first year and third decade of life.2 There is also a well-known association between JXG and neurofibromatosis 1 and acute myelogenous leukemia.10

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