Fatal congenital systemic juvenile xanthogranuloma with liver failure.
This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death at 29 days of age. At autopsy, nodular tumor infiltrates of JXG were present throughout the liver, as well as in skin, abdominal lymph nodes, spleen, and pancreas.
Hu
Weimin K
WK
Department of Dermatology, University Hospitals of Cleveland and Case Western University School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
Gilliam
Anita C
AC
Wiersma
Susan R
SR
Dahms
Beverly B
BB
D000005:Abdomen; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D017093:Liver Failure / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D010179:Pancreas / Q000473:pathology; D012867:Skin / Q000473:pathology; D013154:Spleen / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014972:Xanthogranuloma, Juvenile / Q000150:complications* / Q000151:congenital* / Q000473:pathology