American Journal of Clinical Dermatology

, Volume 11, Issue 1, pp 67–71

Cutaneous Xanthogranulomas, Hepatosplenomegaly, Anemia, and Thrombocytopenia as Presenting Signs of Juvenile Myelomonocytic Leukemia

Case Reports Cutaneous Xanthogranulomas in a Patient with JMML

DOI: 10.2165/11311130-000000000-00000

Cite this article as:
Cham, E., Siegel, D. & Ruben, B.S. AM J Clin Dermatol (2010) 11: 67. doi:10.2165/11311130-000000000-00000


The development of xanthogranulomas has been linked to hematologic malignancies in children and adults, based on a number of reports in the literature. In children, a specific association between juvenile xanthogranuloma, neurofibromatosis 1, and juvenile myelomonocytic leukemia has been described. We report a case of a 9-month-old child, without a known diagnosis of neurofibromatosis 1, who presented with hepatosplenomegaly, anemia, thrombocytopenia, and multiple cutaneous nodules, which were confirmed to be juvenile xanthogranulomas upon biopsy. A concurrent work-up showed that the child had juvenile myelomonocytic leukemia.

Although cutaneous juvenile xanthogranulomas are benign lesions, in several reported cases they have been shown to herald leukemia. This association between xanthogranulomas and hematologic malignancy is poorly understood. Juvenile xanthogranulomas have a number of morphologic variants and clinical presentations that can be confused with the cutaneous lesions of Langerhans cell histiocytosis and dermatofibroma. Recognition of the broad clinicopathologic spectrum of juvenile xanthogranulomas is critical for proper diagnosis.

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© Adis Data Information BV 2010

Authors and Affiliations

  1. 1.Department of PathologyEvanston Northwestern HealthcareEvanstonUSA
  2. 2.Department of Dermatology, Dermatopathology SectionUniversity of CaliforniaSan FranciscoUSA
  3. 3.Department of PathologyUniversity of CaliforniaSan FranciscoUSA

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