histiocytic phagocytic panniculitis
Introduction
Introduction to tissue phagocytic panniculitis In 1980, Winklemann et al first proposed the name of histiocytic pharyngeal pharyngitis (histiocyticphagocytic panniculitis) and reported 5 cases. It is a benign tissue cell proliferative disease characterized by systemic tender multiple subcutaneous nodules, hyperthermia, hepatosplenomegaly, complete blood cell reduction, hemorrhage, and hemagglutination abnormalities. The main feature of histopathology is that tissue cells phagocytose blood components. Forming "bean bag" cells, the disease is caused by infiltration of subcutaneous adipose tissue by tissue cells with phagocytic activity, often involving multiple organs, subcutaneous nodules, fever, whole blood cell reduction, hemorrhage, liver and kidney failure Wait. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.0002% - 0.0004% Susceptible people: no specific population Mode of infection: non-infectious Complications: diffuse intravascular coagulation
Cause
The cause of tissue phagocytic panniculitis
(1) Causes of the disease
The cause of this disease has not yet been determined. It is generally believed that it may be related to hematopoietic diseases or infections. Most scholars believe that the occurrence of this disease is related to microbial infections, especially viral infections. There have been reports of acute EB virus infection, and some people believe that this disease There are many similarities with malignant histiocytosis, which may be considered to be a variant, that is, the extent of the lesion is mainly manifested in malignant histiocytosis of the skin.
(two) pathogenesis
The pathogenesis is still unclear.
1. Some people have used T lymphocyte recombination technology to observe a large number of benign T lymphocytes in patients with subcutaneous lesions. It is believed that this disease may be a reactive disease based on T lymphocyte proliferation, or it is T lymphocyte disease itself, nodularity. Panniculitis, tissue phagocytic panniculitis, malignant histiocytosis, the three have similar clinical symptoms, pathology is expressed as tissue cell proliferation, invasion and phagocytosis, only to varying degrees, between the three Relationships and whether they can be considered as a group of diseases with a spectral distribution are yet to be further studied in the future.
2. For the lobular lipid membrane with focal fatty necrosis, infiltrating cells except for lymphocytes, can be seen morphologically well-differentiated tissue cells, but with obvious phagocytic activity, phagocytic red blood cells, white blood cells and cytoplasm can be seen in the cytoplasm Platelet fragments, which are characteristic of so-called "bean-like" cells, can also be found in visceral tissues such as lymph nodes, liver, spleen, and bone marrow.
Prevention
Tissue cell phagocytic panniculitis prevention
1. Remove infected lesions, pay attention to hygiene, strengthen physical exercise, and improve autoimmune function.
2. The law of life, work and rest, comfortable, avoid strong mental stimulation.
3. Strengthen nutrition, fasting and cold.
4. Early diagnosis, early treatment.
Complication
Tissue phagocytic panniculitis complications Complications, diffuse intravascular coagulation
In the late stage of the disease, there is visceral involvement, complete blood cell reduction, skin purpura or ecchymosis, hepatosplenomegaly, liver and kidney failure, terminal visceral hemorrhage, disseminated intravascular coagulation, infection and other deaths.
Symptom
Tissue cell phagocytic lipid film inflammation common symptoms nodular hepatosplenomegaly hyperthermia scaly subcutaneous nodules intravascular coagulation whole blood cell reduction
Early manifestations of recurrent episodes of tender subcutaneous nodules or plaques, as small as soybeans, as large as the palm, clear boundary, medium hardness, surface is reddish or light brown, can have scales, conscious pain or tenderness, necrosis And ulcers, skin damage can be applied throughout the body, mainly in the abdomen and lower limbs and buttocks, can also be found in the neck, trunk, etc., later may appear purple spots or oral mucosal erosion or ulceration, and Weber-Christian disease damage Very similar, systemic damage is often accompanied by symptoms, may have liver and spleen and lymph nodes, jaundice, serositis, joint pain, often accompanied by fever in the course of the disease, mostly high fever, can cause whole blood cell reduction, liver and kidney dysfunction When the damage is serious in the later stage of development, hepatic and renal failure, the formation of diffuse intravascular coagulation (DIC) and peripheral bleeding, including the digestive tract, respiratory system and urinary system bleeding, often died due to multiple system failure.
Examine
Examination of tissue phagocytic panniculitis
Generally there are hemoglobin, white blood cells and thrombocytopenia, elevated liver enzymes, hypoproteinemia, prolonged prothrombin time, decreased fibrinogen levels, increased circulating fibrin decomposition products, and histopathological changes: such phagocytic cells may also Found in lymph nodes, liver spleen, bone marrow and other visceral tissues, there are a large number of inflammatory cell infiltration in the deep dermis and subcutaneous fat lobules and lobule spaces. The tissue cells are extremely prominent, the cell volume is large, the nucleus is round or kidney-shaped, uniformly stained, and the cytoplasm is rich. Occasionally, nucleoli clusters or scattered, there is a phenomenon of syncytial cells, which contain neutrophils, red blood cells, platelets and nucleolar fragments, forming bean pocket cells, followed by fat cell necrosis, hemorrhage and neutrophil infiltration.
Diagnosis
Diagnosis and identification of tissue phagocytic panniculitis
More common in the lower extremity of the tender subcutaneous nodules, accompanied by fever, hepatosplenomegaly, complete blood cell reduction, histologically visible phagocytic activity of "bean bag-like" cells, can be diagnosed.
Differential diagnosis
1. Nodular panniculitis: the clinical manifestations of the two are similar, mainly from histologically. The histological examination of nodular panniculitis can also be expressed as tissue cell proliferation, phagocytosis and invasion, but only phagocytosis The formation of foam cells, without phagocytizing blood cells to form "bean-like" cells.
2. Malignant histiocytosis: clinically not only subcutaneous nodules, but also skin nodules and papules, tissue cells are abnormally shaped, although there is phagocytosis, but does not form typical "bean-like" cells, The condition is more serious and the course of disease is short.
3. Subcutaneous panniculitis-like T-cell lymphoma: There is tumor cell infiltration in adipose tissue, which is T-cell, moderate tonic nucleus and cerebral nucleus have important diagnostic value, and reactive phagocytic tissue cells may also appear.
