nodular panniculitis
Introduction
Introduction to nodular panniculitis Nodular panniculitis (NP), also known as Weber-Christian disease or relapsing nodular nonsuppurative febrile panniculitis, with recurrent subcutaneous fat inflammatory nodules or plaques A disease characterized by systemic symptoms such as fever, and clinical manifestations are diverse. NP can occur at any age, but it is more common in women aged 30 to 50. The ratio of female to male is about 2.5:1, which is not easy to be recognized. Misdiagnosis. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: sepsis, renal failure
Cause
Cause of nodular panniculitis
Abnormal immune response
Abnormal immune responses can be caused by a variety of antigenic stimuli, such as bacterial infections, foods and drugs. Some cases have been reported to have recurrent tonsillitis before onset, and the disease has been reported in jejunal shunt After that, there are a large number of bacteria in the blind music. In addition, halogen compounds such as iodine, bromine and other drugs, sulfonamides, quinine and expectorants may induce the disease.
Lipodystrophy
Some reports indicate that the disease is associated with abnormalities in certain enzymes during fat metabolism, such as a slight increase in serum lipase or the detection of active pancreatic enzymes and lipases in skin lesions. Some studies have also found that this disease has Alpha-1 anti-trypsin deficiency, of course, this lack of antitrypsin is not likely to directly cause panniculitis, but may lead to dysregulation of immunological and inflammatory responses.
Pancreatic tumor
Pancreatic enzyme is released into the circulation, causing the dissolution and necrosis of distant adipose tissue. The activity of lipase and amylase can be measured in the lesion, and there are reports of coexistence of panniculitis and connective tissue disease. Some may be a kind of LE. Alien.
According to the evolution of the lesion, the pathological changes can be divided into three phases:
1. Degeneration of fat cells in acute inflammatory phase, infiltrated by neutrophils, lymphocytes and tissue cells.
2. Macrophage infiltration is mainly composed of tissue cells, and there are also a small number of lymphocytes and plasma cells.
3. Foam cells are reduced during fibrosis, replaced by fibroblasts, lymphocytes, plasma cells, and finally fibrosis.
Prevention
Nodular panniculitis prevention
Take some medicine according to the doctor's instructions, of course, you need to pay considerable attention to the diet.
Complication
Nodular panniculitis complications Complications sepsis renal failure
People with extensive visceral involvement may die from circulatory failure, hemorrhage, sepsis and renal failure.
Symptom
Nodular lipid inflammatory symptoms common symptoms jaundice loss of appetite intestinal perforation bone marrow inhibition relaxation heat lymph node enlargement liver failure renal failure tissue cell hyperplasia granuloma
Skin damage
Subcutaneous nodules are the main features of NP. The lesions are more common in the extremities, mainly distributed in the femur, buttocks and calves, but the forearms, trunk and face can also appear. The lesions are small subcutaneous nodules at the initial stage of development. Mild bulge, showing erythema and edema, can also be normal skin color; later nodules gradually increase, the boundary is clear, the size is different, the diameter is usually 1 ~ 2cm, the larger diameter can reach more than 10cm; generally 3 ~ 4 , can also be as many as dozens, often symmetrically distributed, into a wholesale, nodules can be slightly moved when deep under the skin; when the position is shallow, it can adhere to the surface of the skin, there is tenderness and spontaneous pain, after several weeks Or after a few months, the nodule subsides by itself, and the local skin is depressed and pigmented. This is due to the occurrence of necrosis, atrophy and fibrosis of the fat in the lesion. The nodules recur every few weeks or months, most episodes There is fever, mostly relaxation heat, the highest can reach 40 ° C, and gradually decline after 1 to 2 weeks, in addition to fever, there may be fatigue, loss of appetite, muscle and joint pain, etc., 10% to 15% of patients No heat, only skin nodules, some nodules will liquefy Degeneration, the skin is also involved and necrotic ulcers, yellow-brown oily liquid outflow, known as liquefied panniculitis (Liquefying panniculitis), is considered to be a variant of NP, the damage mainly occurs in the stock The lower abdomen and the lower abdomen are rare, and 15% of this type has 1 antitrypsin deficiency.
Another variant of NP is called lipogranulomatosis subcutanea, which is Rothmann-Makai syndrome, a specific localized panniculitis that is rare and occurs mainly in children and obesity. In the female, the nodules scattered, no atrophy and depression after the regression, no fever, no systemic symptoms, the entire course of disease 6 to 12 months, and finally self-healing.
2. System damage
Although the disease invades the internal organs and fats, it is rare, but once it occurs, it is mostly involved in the system, and the prognosis is poor. Generally, there are skin lesions and visceral lesions. There are also a few patients with visceral lesions and then typical skin lesions. The diagnosis is more difficult when the system is damaged without skin symptoms. The clinical symptoms of visceral damage depend on the affected organs. The characteristic symptoms are often manifested when the damage is serious. The liver may be affected by right hypochondriac pain, hepatomegaly, jaundice and liver. Abnormal function; small intestinal involvement may have intestinal perforation, fat sputum; mesenteric, omental and retroperitoneal fat tissue involvement may occur in upper abdominal pain, bloating and mass, in addition, bone marrow, lung, pleura, myocardium, pericardium, spleen, Kidney, adrenal gland, brain, etc. can be affected, manifested as myelosuppression and leukopenia, anemia and thrombocytopenia, lung shadow, chest pain and pleurisy, cardiomyopathy and pericarditis, spleen and lymphadenopathy, proteinuria, hematuria and kidney Insufficient function, mental disorder and unconsciousness. People with extensive visceral involvement may die from circulatory failure, hemorrhage, sepsis and renal failure.
There is also a NP variant in systemic damage called cytophagic histiocytic panniculitis (CHP), a benign histiocytosis disease characterized by systemic tender multiple subcutaneous nodules. High fever, hepatosplenomegaly, complete blood cell reduction, abnormal blood coagulation, the disease is critically ill, progressively intensified, the patient's hemorrhagic death is related to diffuse intravascular coagulation and liver failure, histopathology is characterized by "bean bag A cell, a special structure formed by tissue cells phagocytizing other blood cells such as red blood cells.
The disease occurs in young and middle-aged women, subcutaneous nodules occur in batches, nodules have pain and significant tenderness, accompanied by systemic symptoms such as fever, local skin sag after nodular regression, combined with the second stage histopathology Confirmed diagnosis.
Winkelmann and Bowie report a variant of nodular panniculitis, known as histiocytic macrophage panniculitis, a systemic disease characterized by pannicitis, which they believe is Nodular panniculitis endles bleeding, and the patient's bleeding death is related to intravascular coagulation and liver failure. The difference between this disease and nodular vasculitis is that it has abnormal hemorrhagic quality and is in the bone marrow, lymph nodes, liver. Numerous tissue cells appear in the spleen, serosal tissue and subcutaneous fat.
Examine
Nodular panniculitis
1. Significantly increased, white blood cells mildly hyperplasia.
2. Kidney involvement, may have abnormal liver and kidney function, hematuria and proteinuria.
3. Cases may have immunological abnormalities such as decreased complement, increased immunoglobulin, and decreased lymphocyte transformation rate.
4. Involved in anemia, leukopenia and thrombocytopenia.
5. routine examination of increased erythrocyte sedimentation rate, anemia and leukopenia, relative increase in eosinophils, with pancreatic disease, acute blood, elevated urinary amylase, elevated serum lipase, in addition, there are elevated immunoglobulins, Serum complement is reduced and lymphocyte transformation rate is reduced.
6. Pathological examination pathological examination is helpful to confirm the diagnosis, early skin lesions, a large number of inflammatory cell infiltration in adipose tissue, with focal fat necrosis, tissue infiltration in the middle stage, granuloma formation and secondary vascular inflammatory changes In the late stage, fibroblasts separated by small leaflets proliferate, form collagen, replace residual fat tissue, and finally complete fibrosis.
Should be X-ray, B-ultrasound, EEG and brain CT examination.
Diagnosis
Diagnosis and differentiation of nodular panniculitis
diagnosis
The disease is characterized by repeated subcutaneous nodules in the batch, painful and significant tenderness in the nodules, and most of the episodes with fever, combined with the second stage histopathology can be diagnosed.
Differential diagnosis
1. Nodular erythema: good hair in spring and autumn, most of the nodules are distributed on the extension of the calf, symmetry, no rupture, self-resolved after 3 to 4 weeks, no depression at the regression, no visceral damage, mild systemic symptoms, Histopathology indicates inflammation of the interlobular septum.
2.Sweet syndrome: The skin lesions are dark red edematous plaques or nodules in the face, neck and other parts. The boundary is clear, and the surface is especially covered with fake blister-like coarse particles. The touch is hard and tender, and the periphery is hard. Increased white blood cells, especially neutrophils, histopathology showed dermal, central diffuse or tubular neutrophils infiltrated, and nuclear fragmentation.
3. Pancreatitis or pancreatic cancer: The clinical symptoms of subcutaneous nodular fatty necrosis are similar to NP, but the tissue image shows that fat cell necrosis is more serious, accompanied by thick and fuzzy cell wall and non-nuclear shadow cells. , can find the basis of the primary lesion.
4. Subcutaneous lipid inflammatory T-cell lymphoma (SPTCL): SPTCL is a highly malignant T-cell lymphoma, characterized by non-specific subcutaneous nodules with high fever, clinically dangerous, short-term (more than 1 year) Death, histologically characterized by infiltration of tumor cells (CD45RO-positive) between fat leaflets and a large number of bean bag cells (CD68-positive), lesions localized in the subcutaneous tissue, lymph nodes, liver, spleen and other organs outside the skin are not affected, In recent years, with the gradual deepening of the understanding of SPTCL, some scholars have proposed NP cases of repeated fever, unable to heal or even die, which may be SPTCL.
5. Others need to be differentiated from lupus panniculitis (deep lupus) and cutaneous T-cell lymphoma, leprosy and subcutaneous fat necrosis caused by traumatic or foreign body.
