Extramedullary Hematopoiesis of the Cranial Dura and Anhidrotic Ectodermal Dysplasia

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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.

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