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Fatal cytophagic histiocytic panniculitis
Gülten Seçmeer1, Hale Sakalli, Faysal Gök
1Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Abstract:
Individual cases of so-called Weber-Christian disease with a bleeding diathesis have been reported for several years. These were originally diagnosed as Weber-Christian disease, but have been recategorized on review as a chronic, visceral, and cutaneous histiocytic (cytophagic) panniculitis, progressing to liver dysfunction and jaundice and a terminal hemorrhagic diathesis. We report here a rare catastrophic form of systemic panniculitis in an adolescent girl. Despite compelling clinical evidence, the diagnosis was made only on postmortem biopsies.
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