Relapsing fever nodular nonsuppurative panniculitis

Introduction to regression of nodular non-suppurative panniculitis This disease, also known as Weber-Christian syndrome, is an idiopathic panniculitis that mainly affects the abdomen and thigh subcutaneous adipose tissue. The main clinical manifestations are recurrent subcutaneous nodules or plaques, accompanied by Fever, some cases can involve the internal organs. Regression-type nodular non-suppurative panniculitis with multiple system damage, called systemic panniculitis; only skin lesions without visceral damage, called common type of panniculitis. basic knowledge The proportion of illness: 0.0007% Susceptible people: no specific population Mode of infection: non-infectious Complications: meningitis, rheumatic fever, cardiomyopathy, intestinal obstruction, cirrhosis, myelofibrosis, retroperitoneal fibrosis

Regression of the cause of thermal nodular non-suppurative panniculitis

(1) Causes of the disease

It is not very clear, it is generally believed that it may be a non-specific reaction caused by various reasons, or an infection, a drug-induced allergic disease. It is reported that there are recurrent tonsillitis before the onset of the disease. It is reported that there are different cases of tuberculosis factors in some cases, and some cases must be treated with anti-tuberculosis treatment before the disease can be relieved. Halide, sulfonamide, quinine and other drugs may induce panniculitis, dermatomyositis, systemic lupus erythematosus, thus This disease is associated with autoimmune rheumatism.

(two) pathogenesis

Fat granuloma (27%):

Fat granuloma is a granuloma reaction caused by a variety of factors causing degeneration of adipose tissue. These factors are divided into local and systemic. Local factors are transient ischemia, small arterial spasm, and systemic factors may be lipodystrophy. Some factors cause abnormalities in certain enzymes during fat metabolism, such as serum amylase, increased lipase, local lipase, and elevated proteases, which result in damage to fat cells, which ultimately leads to the formation of fatty granuloma.

Pathological examination (15%):

The main lesions in the fat layer are generally divided into three phases: (1) acute inflammatory phase: fat cell degeneration and intercellular inflammatory infiltration, including neutrophils, lymphocytes and histiocytes, mainly neutrophils, and Common neutrophil nucleus destruction, but no abscess formation. (2) Macrophage phase: Mainly infiltrated by tissue cells, phagocytized fat and become foamy macrophages, with a small amount of lymphocytes, plasma cells and neutrophils infiltrated, often with multinucleated giant cells. (3) Fibroblast phase: The lesions are mainly fibroblasts and lymphocytes, and have a large number of hyperplastic fibrous tissues.

Lesion (10%):

In the process of lesion evolution, small blood vessels also changed, mainly in perivascular cell infiltration, intimal thickening and fibrin-like degeneration, omentum, mesentery, pericardium, liver, spleen, bone marrow and adrenal fat. The same pathological changes, diffuse fat necrosis and fat infiltration in the liver, lung pathological changes in the alveolar cavity and interstitial inflammatory cell infiltration, granulomatous pneumonia and fat embolism.

Regression of heat type nodular non-suppurative 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, pay attention to warming.

Regression of heat-type nodular non-suppurative panniculitis Complications meningitis rheumatic fever cardiomyopathy intestinal obstruction cirrhosis myelofibrosis peritoneal fibrosis

Occasionally, pericarditis can occur, heart failure can occur in the later stages of the disease, central nervous system, meningitis symptoms and intracranial hypertension can occur, the disease can be associated with rheumatoid arthritis, rheumatic fever, ulcerative colitis and systemic lupus erythematosus Overlap, in addition to glomerulonephritis, can occur complications such as cardiomyopathy, coronary occlusion, granulomatous pneumonia, intestinal obstruction, cirrhosis, myelofibrosis and retroperitoneal fibrosis.

Regression of hot nodular non-suppurative lipid film symptoms Common symptoms Difficulty vesicular joint swelling and pain Joint pain High heat Irregular fever Anorexia nausea abdominal pain Upper abdominal pain

Most cases are accompanied by headaches, generalized weakness, anorexia, muscle pain, joint swelling and pain and mental discomfort.

1. The general performance is found in common and system types.

(1) Fever: It is more common in system type. It starts to heat up after a few days of skin lesions, and the body temperature gradually rises. It can be as high as 40 °C or more, and it has a relaxation heat. After 1, 2 weeks, the body temperature begins to decrease, except for relaxation. In addition to heat, it can also be intermittent heat and irregular heat.

(2) Skin lesions: occur in the limbs and torso, most commonly in the buttocks and thighs. The lesions are solid subcutaneous nodules that occur in batches, or in pieces, varying in size, as small as peas, as large as palms. The edge is clear, the nodules can adhere to the skin, the surface is reddish, with mild tenderness and tenderness. A few nodules can be necrotic and ruptured, and the fatty substances flow out, but no pus, occasionally the skin lesions can be expressed as blisters, subcutaneous nodules After a few days or weeks, it can gradually disappear, and the affected area is slightly sunken or brown pigmented.

(3) joints: manifested as joint pain, the most common knee pain, followed by wrist joints, ankle joints, and some cases can also show migratory joint pain.

(4) Others: Some patients may have lymphadenopathy, which is more common in system type, mostly located under the armpit, inguinal and tracheal, 0.5 to 2 cm in diameter. Some patients may have edema, lower extremity edema, orbital edema or systemic edema. .

2. Visceral involvement is only seen in the system type.

(1) Respiratory system: Individual patients have chest pain, dyspnea symptoms, physical examination and vesicle sounds, pleural friction sounds, and occasional signs of exudative pleurisy.

(2) Digestive system: can occur anorexia, nausea, abdominal pain, diarrhea, jaundice, gastrointestinal bleeding and liver, splenomegaly, lesions such as involving mesentery, omentum, posterior peritoneum and pelvic adipose tissue, can cause upper abdominal pain, tenderness And intestinal peristalsis, auscultation shows weakening of bowel sounds, this condition is called abdominal panniculitis, often accompanied by high fever, abdominal pain, weight loss, etc., abdominal fatitis can cause intestinal obstruction due to fibrosis.

(3) cardiovascular system: can show myocarditis, cardiac hypertrophy, tachycardia, occasionally can also occur pericarditis, heart failure can occur later in the course of the disease.

(4) Eye damage: Some cases may have ocular symptoms, such as anterior uveitis, acute exudative choroiditis and secondary glaucoma.

(5) Central nervous system: can be expressed as mental disorders, disturbance of consciousness, coma, convulsions, meningitis symptoms and intracranial hypertension, these symptoms are caused by intracranial adipose tissue inflammation.

(6) overlap with other rheumatism: this disease can overlap with rheumatoid arthritis, rheumatic fever, ulcerative colitis and systemic lupus erythematosus, in addition to glomerulonephritis.

It must be pointed out that there is no absolute limit between the ordinary type and the system type, and the ordinary type can develop into a system type as the disease progresses.

Regression of thermal nodular non-suppurative panniculitis

1. Blood routine, bone marrow and blood sedimentation of peripheral blood can be seen in red blood cells, thrombocytopenia, white blood cells are mostly reduced, can contain poisoning particles, neutropenia with differential count, with left nucleus, but increased white blood cells and increased erythrocyte sedimentation rate The bone marrow can be seen as granulocytes, red blood cells and megakaryocytes are reduced to varying degrees. Sometimes granulocytes can contain a few toxic particles, which are infected with bone marrow.

2. Urine routine When combined with glomerulonephritis or overlapping other rheumatism, proteinuria, hematuria and tubular urine may occur.

3. Biochemical examination Liver involvement may have abnormal liver function and increased CRP.

4. Immunological examination of some patients with anti-"O" titer increased, overlap with other rheumatism may have increased rheumatoid factor titer, anti-nuclear antibody positive, complement decreased, IgG, IgM increased, low cellular immunity test, Lymphocyte conversion rate is reduced.

5. X-ray examination of the chest radiograph showed hilar and paratracheal lymph node enlargement, occasionally a small amount of pleural effusion, increased texture and spotting in the lungs, involving the cardiovascular system, showing heart enlargement and heart failure .

6. ECG examination showed sinus tachycardia, different types of conduction block and myocarditis.

7. B-ultrasound images show subcutaneous nodules, which are characterized by thickening of the subcutaneous space at the edge of the lesion and the diffuse parenchymal structure.

Retrospective diagnosis of differential nodular non-suppurative panniculitis

Ordinary type is based on repeated episodes of subcutaneous nodules or flaky plaques, accompanied by fever, and localized depression after spontaneous regression of skin lesions. It is not difficult to diagnose. The final diagnosis depends on biopsy. Systematic diagnosis is complicated. In addition to the general manifestations of fever, subcutaneous nodules, and other organ manifestations, individual patients have neither fever nor subcutaneous nodules, only manifested as symptoms of various organs, requiring biopsy to be clear diagnosis.

Differential diagnosis

(1) nodular erythema: skin lesions involving the dermis and subcutaneous tissue, caused by skin necrotizing vasculitis, occur in the bilateral calves, leaving only mild pigmentation after erythema subsides, leaving no local depression, The histological changes in lesions are vasculitis, not a change in adipose tissue inflammation.

(2) Hard erythema: skin lesions occur in the bilateral calf flexion, no systemic symptoms such as fever, histopathology is tuberculosis, and anti-tuberculosis treatment is effective.

(3) Skin type: malignant reticulocyte disease has a hard skin texture. After ulceration, it can form a deep ulcer with clear edges, covered with secretions and thick black sputum. In some cases, there may be a history of nasopharynx tumors. Blood decline and progressive liver, spleen, lymph node enlargement, histopathological changes to reticulocyte hyperplasia, hyperplastic reticulocytes are not typical or immature.

(4) multiple neurofibromatosis: an autosomal dominant hereditary disease with multiple subcutaneous soft tissue tumors, multiple fibroids distributed along the nerve trunk, and skin pigmentation, involving the peripheral nerves And the central nervous system, causing the corresponding clinical manifestations, the pathological features of which are soft fibroids and fibroids.

(5) Lymphoma: manifested as fever, systemic lymphadenopathy, sometimes accompanied by joint muscle discomfort, hepatosplenomegaly, etc., pathological changes of enlarged lymph nodes are abnormal lymphocyte hyperplasia, granulomatousness seen without liposuitis Adipose tissue is inflammatory.

2. System type should be identified with the following diseases

(1) rheumatoid arthritis: general onset of acute disease, mainly polyarthritis, accompanied by fever and leukocytosis, many degrees of carditis, histological examination of subcutaneous nodules can be seen Aschoff (Aschoff) body It is effective for the treatment of salicylic acid preparations.

(2) Systemic lupus erythematosus: it has multiple lesion characteristics and is easily confused with the disease, but its skin characteristics and histological changes are significantly different from the disease. Furthermore, immunological examination and anti-nuclear antibodies are positive. The characteristic lesions found in panniculitis indicate that systemic lupus erythematosus overlaps with the disease.

(3) Pancreatic cancer has severe gastrointestinal symptoms, such as nausea, vomiting, weight loss, etc. When the common bile duct is compressed, it can cause progressive deepening of jaundice. B-ultrasound and CT examination can reveal signs of pancreatic cancer, sometimes pancreatic cancer. It may be associated with nodular liquefaction panniculitis, but the histological changes of the lesion are different from the disease, and serum amylase and lipase are elevated.

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