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DOI: 10.1055/s-2008-1071322
© Georg Thieme Verlag KG Stuttgart · New York
Extramedullary Hematopoiesis of the Cranial Dura and Anhidrotic Ectodermal Dysplasia
Publication History
Publication Date:
19 March 2008 (online)
Abstract
A 2-year-old amerasian male with anhidrotic ectodermal dysplasia (Christ-Siemens-Touraine Syndrome) was admitted for status epilepticus and Mycobacteria avium-intracellulare infection. A computed tomography scan of the head revealed a mass thought to be a subdural hematoma. The patient died following overwhelming Mycobacteria avium-intracellulare and Pseudomonas aeruginosa sepsis. Autopsy revealed extensive extramedullary hematopoiesis of the dura forming a tumor-like thickening with focal subdural hemorrhage. To our knowledge, this is the first report of extramedullary hematopoiesis of the cranial dura associated with anhidrotic ectodermal dysplasia.
Key words
Dura - Ectodermal dysplasia Extramedullary hematopoiesis - Immunodeficiency