A Case of Multiple Juvenile Xanthogranuloma Involving Skin and Systemic Organs / 소아과

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.