LAUSR

A 2-mo-old girl had hepatosplenomegaly and liver dysfunction (direct bilirubin level: 12.2 mg/dl, aspartate transaminase level: 202 IU/L, alanine aminotransferase level: 147 IU/L) with ascites since birth (Fig. 1a). When the patient developed brown rashes on her face at the age of 2 mo (Fig. 1b), skin and liver biopsies were performed. The skin biopsy showed infiltration of histiocytes with a foamy cytoplasm. The liver was infiltrated by histiocytes with a foam-like cytoplasm (Fig. 2a). The histiocytes were CD163-positive (Fig. 2c) and CD1aand S-100-negative. Additionally, there were granulomas with Touton giant cells (Fig. 2b). These results did not match the findings of immunohistochemistry of Langerhans cell histiocytosis (LCH), which was CD1a-, langerin-, and S-100-positive. Finally, she was diagnosed as having disseminated juvenile xanthogranuloma (JXG) and then prednisolone was started. Despite the therapy, liver failure became worse, and the patient died from sepsis at the age of 2 mo. The autopsy findings were compatible with histological manifestation of JXG, and showed similar CD163-positive cell infiltration in the lungs, liver, kidneys and spleen. JXG is a histiocytic tumor that usually appears at birth or during the first year of life [1]. Disseminated JXG accounts for approximately 5% of skin type JXG [2]. Although the optimal treatment for disseminated JXG has not been established yet [3], it is usually treated according to the treatment for LCH [4]. However, there have been some cases of natural healing of JXG [5]. Distinctive skin lesions and histopathological findings suggest the diagnosis of disseminated JXG, but they are sometimes not evident at the onset of symptoms [6]. Dehner reported that the characteristic Touton giant cell was a consistent feature of cutaneous lesions