Fatal cytophagic histiocytic panniculitis.
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.
Seçmeer
Gülten
G
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Sakalli
Hale
H
Gök
Faysal
F
Ozen
Seza
S
Kara
Ates
A
Cengiz
Ali Bülent
AB
Ceyhan
Mehmet
M
Gürgey
Aytemiz
A
Gögüs
Safiye
S
Kanra
Güler
G
D000293:Adolescent; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D010201:Panniculitis, Nodular Nonsuppurative / Q000473:pathology*