Cytophagic histiocytic panniculitis

In 1980, Winklemann et al. first proposed the name histiocytic phagocytic panniculitis and reported 5 cases. It is a benign histiocytic proliferative disease characterized by generalized tender multiple subcutaneous nodules, high fever, hepatosplenomegaly, pancytopenia, bleeding and abnormal blood coagulation; the main histopathological feature is histiocytes phagocytosing blood components to form bean-bag cells. This panniculitis is caused by infiltration of subcutaneous adipose tissue by phagocytically active histiocytes, often involving multiple organs, with subcutaneous nodules, fever, pancytopenia, bleeding, and liver and kidney failure.

Common Symptoms

Intravascular coagulation; scales; nodule; hepatosplenomegaly

Causes

(1) Etiology. The cause remains undetermined; it is generally thought to relate to hematopoietic disease or infection. Most scholars link it to microbial infection, especially viral infection; there are reports combined with acute EB virus infection, and some note many similarities to malignant histiocytosis, suggesting it may be a variant, i.e. malignant histiocytosis mainly manifesting in the skin. (2) Pathogenesis. The mechanism is unclear. 1. Using T-lymphocyte recombinant technology, a large number of benign T lymphocytes were observed in subcutaneous lesions, suggesting it may be a reactive disease based on T-lymphocyte proliferation, or itself a T-lymphocyte disease. Nodular panniculitis, cytophagic histiocytic panniculitis and malignant histiocytosis have similar clinical features, all showing histiocyte proliferation, invasion and phagocytosis, differing only in degree; the relationship and whether they form a spectral group await further study. 2. It is lobular panniculitis with focal fat necrosis; besides lymphocytes, well-differentiated histiocytes with marked phagocytic activity are seen, with phagocytosed red cells, white cells and platelet fragments in the cytoplasm forming characteristic bean-bag cells; such phagocytes are also seen in lymph nodes, liver, spleen and bone marrow.

Treatment

Medication, supportive treatment

Prevention

1. Remove infectious foci, maintain hygiene, strengthen exercise and improve immune function. 2. Live regularly, balance work and rest, stay in a good mood, and avoid strong mental stimulation. 3. Strengthen nutrition and avoid raw and cold food. 4. Diagnose and treat early.

Recommended Department

It is recommended to visit the rheumatology and immunology department; before the visit you may first book an appointment and connect with experts through this platform.

Warm reminder: The above information is provided for health education reference only and cannot replace professional medical diagnosis.