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A 12-year-old boy is referred for evaluation of a skin lesion located on his right inner thigh. The growth has been present for several months and has remained asymptomatic. The patient is in good health and has no similar lesions elsewhere on the body. The family history is negative for genodermatoses. Examination of the lesion reveals a reddened 1.0cm firm nodule.
Juvenile xanthogranuloma (JXG) is a skin disorder of non-Langerhans cell histiocytes that presents during infancy or childhood. The condition is a granulomatous histiocytic response thought to be triggered by a physical or infectious stimulus.1 Juvenile xanthogranuloma characteristically presents as an asymptomatic...
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Juvenile xanthogranuloma (JXG) is a skin disorder of non-Langerhans cell histiocytes that presents during infancy or childhood. The condition is a granulomatous histiocytic response thought to be triggered by a physical or infectious stimulus.1 Juvenile xanthogranuloma characteristically presents as an asymptomatic papulonodular lesion that may be red, brown, or yellow in coloration. Multiple lesions are found in approximately 7% of cases.2
Juvenile xanthogranuloma rarely occurs outside of the skin with the eye being the most common noncutaneous site.3 It has been reported that JXG can be found in association with neurofibromatosis and juvenile myelomonocytic leukemia.2,4
Dermoscopic visualization of JXG reveals a setting sun appearance consisting of a red-yellow center surrounded by a discrete erythematous halo.5 Definitive diagnosis of JXG is made by biopsy, which characteristically reveals Touton giant cells and lipid-laden histiocytes accompanied by a mixed cellular infiltrate containing histiocytes, lymphocytes, eosinophils, neutrophils, and plasma cells.6 The majority of cases spontaneously regress within 1 to 5 years. Surgical excision is curative.
Sidney Lampert is a medical student at the Drexel University College of Medicine, in Philadelphia. Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Szczerkowska-Dobosz A, Kozicka D, Purzycka-Bohdan D, Biernat W, Stawczyk M, Nowicki R. Juvenile xanthogranuloma: a rare benign histiocytic disorder. Postepy Dermatol Alergol. 2014;31(3):197-200. doi:10.5114/pdia.2014.40918
2. Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003;27(5):579–593. doi:10.1097/00000478-200305000-00003
3. Meyer P, Graeff E, Kohler C, Munier F, Bruder E. Juvenile xanthogranuloma involving concurrent iris and skin: clinical, pathological and molecular pathological evaluations. Am J Ophthalmol Case Rep. 2017;9:10-13. doi:10.1016/j.ajoc.2017.09.004
4. Meyer SN, Vaughn A, Li Y, Studer AC, Rauen KA, Kiuru M. The association between juvenile xanthogranulomas in neurofibromatosis type 1 patients and the development of leukaemia: a systematic review. J Eur Acad Dermatol Venereol. 2023;37(12):e1380-e1383. doi:10.1111/jdv.19321
4. Oliveira TE, Tarlé RG, Mesquita LAF. Dermoscopy in the diagnosis of juvenile xanthogranuloma. An Bras Dermatol. 2018;93(1):138-140. doi:10.1590/abd1806-4841.20186849
5. Vahabi-Amlashi S, Hoseininezhad M, Tafazzoli Z. Juvenile xanthogranuloma: case report and literature review. Int Med Case Rep J. 2020 Feb 25;13:65-69. doi:10.2147/IMCRJ.S240115
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