Cytophagic histiocytic panniculitis - a syndrome associated with benign and malignant panniculitis: Case comparison and review of the literature

Many cases of hemophagocytic syndromes with cytophagic histiocytic panniculitis (CHP) are being classified and considered as a natural disease progression of subcutaneous panniculitic T-cell lymphoma (SPTL). Review of the literature on both CHP and SPTL discloses distinct patterns suggesting these disorders may not be equivalent, even though a terminal hemophagocytic syndrome may be associated with each. Some SPTL appear to have association with Epstein-Barr virus (EBV), show malignant histopathology in the skin, and may disseminate terminally, with a majority of cases showing a rapidly fatal progression. On the other hand, classic cases of CHP without proven lymphoma may not be associated with EBV, appear to be histologically benign, and have an indolent course unless terminal hemophagocytic syndrome develops. We compared and contrasted a case of CHP and a case of SPTL and reviewed the literature. Our observations suggest that the often fatal hemophagocytic syndrome may be associated with both benign and malignant subcutaneous panniculitis.