Cytophagic Histiocytic Panniculitis with Encephaloclastic Changes: A Case Report and Literature Review

摘要

Cytophagic histiocytic panniculitis (CHP) was first described by Winkelmann and Bowie in 1980. It is a rare group of diverse illnesses involving benign and malignant proliferation of macrophages in various organs and tissues. It presents with subcutaneous panniculitis with or without a hemophagocytic syndrome (HPS). It occurs predominantly in women (male: female ratio 1:1.3) between the years of 5～61 (average, 33.5). The major clinical features are recurrent fever, multiple panniculitic lesions, anemia, leukopenia and coagulation abnormalities. In the later phase, liver dysfuction, serosal effusion, mucosal ulceration and hemorrhage may occur. Histological findings show activated histiocyte infiltration of the fat tissue. Cytologically the benign-looking histiocytes containing cell fragments (bean-bag cells) are very typical. CHP has a broad spectrum from mild to severe. Benign CHP is selflimiting and sensitive to treatment, but up to now there is no effective therapy for malignant CHP. We report here a case of progressive and fatal cytophagic histiocytic panniculitis in a young woman who had encephaloclastic changes immediately prior to her death.