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A Case of Multiple Juvenile Xanthogranuloma Involving Skin and Systemic Organs

Korean Journal of Pediatrics 2004;47(2):227-230.
Published online February 15, 2004.
A Case of Multiple Juvenile Xanthogranuloma Involving Skin and Systemic Organs
So Yeon Lee1, Yong Seok Jang1, Min Sun Kim1, Yun Jeong Yang1, Myoung Ja Jung2, Pyoung Han Hwang1
1Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea
2Department of Pathology, Chonbuk National University Medical School, Jeonju, Korea
피부와 전신장기를 침범한 다발성 연소성 황색육아종 1례
이소연1, 장용석1, 김민선1, 양윤정1, 정명자2, 황평한1
1전북대학교 의과대학 소아과학교실
2전북대학교 의과대학 해부병리학교실
Correspondence: 
Pyoung Han Hwang, Email: hwaph@moak.chonbuk.ac.kr
Abstract
Juvenile xanthogranuloma(JXG) is one of the most common non-Langerhans cell histiocytosis, benign histiocytic proliferative disorder. It typically presents as a solitary, benign, rapidly growing cutaneous tumor that may regress spontaneously. Most cutaneous lesions of JXG occur in the head and neck region of infants and young children. This disease is usually present with cutaneous lesion only; visceral involvement including the lung, bone, testis, gastrointestinal tract, kidney, heart, eye and oral cavity is rare, but may affect various organs. Moreover, JXG is associated with neurofibromatosis type I and juvenile chronic myeloid leukemia. In contrast to the cutaneous form, systemic JXG may be associated with significant complications requiring aggressive medical care. The authors report a case of JXG in a 3-month-old male infant which involved systemic organs, with a brief of the literature. The patient presented with cutaneous, pulmonary, pancreatic, testicular and hepatic nodules. The cutaneous nodule biopsy shows the proliferation of histiocytes which have indistinct borders, round vesicular nuclei, foamy cytoplasm and eosinophils. The patient's follow-up was characterized by slow and progressive clinical improvement without specific treatment.
Key Words: Juvenile xanthogranuloma, Systemic involvement


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