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Fatal Congenital Systemic Juvenile Xanthogranuloma with Liver Failure

  • Case Report
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Pediatric and Developmental Pathology

Abstract

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.

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Acknowledgments

We gratefully acknowledge the help of Louis P. Dehner, MD (Department of Pathology, Washington University, St. Louis, MO), who consulted on this patient and performed immunohistochemistry for factor XIIIa.

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Authors and Affiliations

  1. Department of Dermatology, University Hospitals of Cleveland and Case Western University School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA

    Weimin K. Hu & Anita C. Gilliam

  2. Department of Pediatrics, University Hospitals of Cleveland, 11100 Euclid Avenue, Cleveland, OH 44106, USA

    Susan R. Wiersma & Beverly B. Dahms

Authors
  1. Weimin K. Hu
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  2. Anita C. Gilliam
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  3. Susan R. Wiersma
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  4. Beverly B. Dahms
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Correspondence to Beverly B. Dahms.

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Hu, W., Gilliam, A., Wiersma, S. et al. Fatal Congenital Systemic Juvenile Xanthogranuloma with Liver Failure . Pediatr. Dev. Pathol. 7, 71–76 (2004). https://doi.org/10.1007/s10024-003-4040-3

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  • DOI: https://doi.org/10.1007/s10024-003-4040-3

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