nodular panniculitis in children
Introduction
Introduction to children with 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. The disease was first reported by Pfeifer in 1882. In 1952, Weber described the recurrence and non-suppurative features of the disease. In 1928, Christian emphasized the febrileness. Some cases have no fever, and similar reports have been reported in China. The disease is not uncommon. Because of its diversified clinical manifestations, NP can occur at any age, but it is more common in women aged 30-50 years. The ratio of female to male is about 2.5:1, which is difficult to be recognized and often misdiagnosed. basic knowledge The proportion of sickness: 0.0001% - 0.0002% Susceptible people: children Mode of infection: non-infectious Complications: epilepsy
Cause
Causes of nodular panniculitis in children
(1) Causes of the disease
The cause is not known and may be related to the following factors:
1. Associated with lipodystrophy: It has been reported that this disease is associated with abnormalities in certain enzymes in the fat process, such as a slight increase in serum lipase or the detection of active trypsin and lipase in the lesion.
2. Associated with allergies: can be caused by a variety of antigenic stimuli, such as bacterial infections, foods, drugs (such as halogen compounds, sulfonamides, quinine and tinctures).
3. Accompanying autoimmune disease: This disease can be associated with diseases such as lupus erythematosus, scleroderma, dermatomyositis or nodular polyarteritis.
(two) pathogenesis
Some people think that adult cases are closely related to pancreatitis and pancreatic tumors. Pancreatic enzymes are released into the circulation, causing the dissolution and necrosis of distant adipose tissue. The activity of lipase and amylase can be measured in the lesions, as well as panniculitis and connective tissue. Some reports of coexisting tissue diseases may be a variant of LE.
According to the evolution of the lesion, the pathological changes can be divided into three phases:
1. Acute inflammatory phase: Degeneration of fat cells, infiltrated by neutrophils, lymphocytes and tissue cells.
2. Macrophage phase: Infiltration is mainly composed of tissue cells, and there are also a small number of lymphocytes and plasma cells.
3. Fibrosis stage: Foam cells are reduced, replaced by fibroblasts, lymphocytes, plasma cells, and finally fibrosis.
Prevention
Childhood nodular panniculitis prevention
The cause of this disease is unknown, and predisposing factors such as infection, drugs, and trauma should be prevented.
Complication
Pediatric nodular panniculitis complications Complications
Hepatosplenomegaly, when the bone marrow is involved, the whole blood cells are reduced, which can cause epilepsy.
Symptom
Nodular lipid film inflammation in children Common symptoms Whole blood cell reduction Subcutaneous nodules Skin adhesions Thin joint pain Painful meningitis Hepatosplenomegaly
Skin damage
In the lower back, the femur, the buttocks, the upper arm, the abdomen, and the face part of the batch appear 1 to 2 cm in diameter, skin color or slightly red subcutaneous nodules, some can be fused into plaque, mostly symmetrical distribution, children with facial hair Disseminated subcutaneous pain nodules are characterized by more or less nodules or plaques, firm texture, slight movement or adhesion to the skin, mild tenderness, and softening and absorption of skin lesions after a few weeks. The subcutaneous fat is atrophied, forming a disc-shaped skin depression, a few nodules can be broken, and a yellow or brown oily liquid flows out.
2. Systemic symptoms
Whenever a new group of skin lesions appears, there are all body manifestations, such as fever, discomfort, hepatosplenomegaly, weight loss, joint pain and so on.
3. Organ involvement
In some cases, in addition to subcutaneous nodules, internal organs, such as lung infiltration, hepatic steatosis, bone marrow involvement, whole blood cell reduction, exudative serositis, meningeal fat lesions, epilepsy and neurological symptoms, mesenteric , omental and retroperitoneal fat involvement, upper abdominal pain and gastrointestinal symptoms.
Examine
Examination of nodular panniculitis in children
1. routine inspection
Increased erythrocyte sedimentation rate, anemia and leukopenia, relative increase in eosinophils, pancreatic disease, acute blood, elevated urinary amylase, elevated serum lipase, in addition, elevated immunoglobulin, serum complement, Lymphocyte conversion rate is reduced.
2. Pathological examination
Pathological examination helps to confirm the diagnosis, early skin lesions, a large number of inflammatory cell infiltration in adipose tissue, focal fat necrosis, tissue infiltration in the middle stage, granuloma formation and secondary vascular inflammatory changes, later by leaflets Interstitial fibroblasts proliferate, form collagen, replace residual fat tissue, and finally complete fibrosis.
3. Should be X-ray, B-ultrasound, EEG and brain CT examination.
Diagnosis
Diagnosis and differential diagnosis of nodular panniculitis in children
diagnosis
According to the above clinical manifestations and laboratory tests, the symptoms are not difficult to diagnose, and if necessary, skin biopsy is confirmed.
Differential diagnosis
Bone marrow involvement should be differentiated from aplastic anemia, and pulmonary infiltration should be differentiated from pulmonary inflammatory lesions.
