Fatal cytophagic histiocytic panniculitis

Secmeer G., Sakalli H., Gok F., Ozen S., Kara A., Cengiz A., ...Daha Fazla

PEDIATRIC DERMATOLOGY, cilt.21, ss.246-249, 2004 (SCI İndekslerine Giren Dergi) identifier identifier identifier


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.