Fatal cytophagic panniculitis and haemophagocytic syndrome.
We present two fatal cases of cytophagic panniculitis (CP) and haemophagocytic syndrome (HPS). In the first of these, there was an underlying T-cell lymphoma but in the second no associated disease was found. HPS is a frequently fatal disorder of immune regulation, characterized by fever, histiocytic haemophagocytosis, hepatosplenomegaly, pancytopenia, hypertriglyceridaemia and coagulopathy; CP is a less common manifestation. A number of benign and malignant conditions may present with HPS, the clinical findings and investigations aiding in determining an underlying disorder. Therapy is both supportive and directed at any associated illness, but often very difficult as diagnosis is delayed.
St John's Institute of Dermatology, London, UK.
S C
SC
Huilgol
D
D
Fenton
H
H
Pambakian
R
R
Cerio
D M
DM
McGibbon
M M
MM
Black
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D016399:Lymphoma, T-Cell / Q000150:complications; D008297:Male; D015434:Panniculitis / Q000175:diagnosis* / Q000209:etiology; D010257:Paraneoplastic Syndromes / Q000209:etiology