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Cytophagic histiocytic panniculitis: A diagnostic dilemma
Author(s) -
Cheah PhaikLeng,
Looi LaiMeng,
Tan PuayEng,
Bosco John,
Kuperan Ponnudurai
Publication year - 1992
Publication title -
hematological oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 44
eISSN - 1099-1069
pISSN - 0278-0232
DOI - 10.1002/hon.2900100606
Subject(s) - medicine , panniculitis , pancytopenia , histiocyte , dermatology , hemophagocytosis , pathology , rare disease , disease , bone marrow
Cytophagic histiocytic panniculitis (CHP) is a recently recognized entity that frequently poses a perplexing diagnostic problem. Although the classical case presents with a relapsing fever, subcutaneous nodules, pancytopenia and liver dysfunction, most patients have in addition a multitude of other manifestations which confuse the clinical picture. Notwithstanding the variable clinical course, the disease frequently terminates in fatal hemorrhage. Diagnosis is based on histological features. A lobular panniculitis with an infiltrate of cytologically benign cytophagocytic histiocytes in skin nodules is the sine qua non of CHP. Hence, a deep skin biopsy which includes subcutaneous fat is mandatory to establish the diagnosis. Published information regarding this newly described entity remains scarce and we report two cases of CHP, one occurring in a 30‐year‐old Kadazan man and another in a 17‐year‐old Chinese woman seen at the University Hospital, Kuala Lumpur. The latter case presented with exudative ascites, an unusual feature, possibly due to intra‐abdominal panniculitis. In addition, we record the development of cirrhosis in the same patient.