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An Atypical Presentation of Juvenile Xanthogranuloma

Updated: Apr 19, 2019

Michael Ryan, Dr. Adam V Nguyen, Dr. Matthew J Hay, Dr. Brent C Kelly, Dr. Sharon S Raimer

University of Texas Medical Branch

Juvenile xanthogranuloma (JXG) is an uncommon benign histiocytic tumor that most commonly appears in children during the first year of life.[1,2] It is the most common of the cutaneous and mucocutaneous histiocytoses, but the exact incidence and prevalence are unknown.[2] Lesions are often present at birth or develop in the first years of life and regress over time. JXG may increase in size or number, but typically become stable or begin to spontaneously regress and disappear by the age of three to six years.[1] The disease is generally asymptomatic, self-limiting, and has an excellent prognosis. Very rarely JXG can have visceral or ocular involvement which can lead to morbidity and mortality.[3] We present the case of a 5-week-old male who was brought to the dermatology clinic for evaluation of nodules under the skin of his scalp, trunk, groin, and extremities, many of which had a blue hue to the overlying skin. The infant had an uncomplicated delivery at which time he was noted to have thrombocytopenia with petechiae and bruising covering much of his body. A biopsy of one of these blue nodules was performed in the dermatology office and histologic examination supported the diagnosis of JXG. In the majority of cases, JXG presents as a single, well-demarcated, dome-shaped cutaneous papule or nodule with red, orange, yellow or brown coloring.[1,4] This case of JXG presenting as multiple subcutaneous nodules with unusual overlying blue hue represents a highly atypical presentation of an uncommon dermatologic disorder.

References: [1]. Gianotti F, Caputo R. Histiocytic syndromes: a review. Journal of the American Academy of Dermatology. 1985 Sep 1;13(3):383-404. [2]. Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB. Juvenile xanthogranuloma. Journal of the American Academy of Dermatology. 1997 Mar 1;36(3):355-67. [3]. Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP. Juvenile xanthogranuloma: forms of systemic disease and their clinical implications. The Journal of pediatrics. 1996 Aug 1;129(2):227-37. [4]. Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. The American journal of surgical pathology. 2003 May 1;27(5):579-93.

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