Extramedullary hematopoiesis of the cranial dura and anhidrotic ectodermal dysplasia.
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-intracellular 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.
Sitton
J E
JE
Department of Pathology, Tulane University School of Medicine, New Orleans, Louisiana 70112.
Reimund
E L
EL
D000361:Agammaglobulinemia / Q000235:genetics* / Q000473:pathology; D002675:Child, Preschool; D004388:Dura Mater / Q000473:pathology*; D004439:Eccrine Glands / Q000473:pathology; D004476:Ectodermal Dysplasia / Q000235:genetics* / Q000473:pathology; D006411:Hematopoiesis, Extramedullary / Q000235:genetics*; D006412:Hematopoietic Stem Cells / Q000473:pathology; D006801:Humans; D008297:Male; D013154:Spleen / Q000473:pathology