OVERVIEW
Nodular lipomatitis (NP), also known as regressive febrile nodular nonsuppurative lipomatitis, is a disease characterized by recurrent episodes of inflammatory nodules or plaques in the subcutaneous fat layer, accompanied by fever and other systemic symptoms. The disease was first reported in 1882 and then described in 1952 as having recurrent, nonsuppurative, febrile features, so it is also known as recurrent nodular lipomatitis or Weber-Christian syndrome. The disease is an inflammatory condition that originates in the fat layer and may involve the viscera, peritoneum, and greater omentum in addition to the subcutis, resulting in multiple organ damage. The disease is not rare and can occur at any age, but is more common in women between 30 and 50 years of age, with a female-to-male ratio of about 2.5:1. It can also occur at other ages, even in infants and young children. The disease can also occur at other ages, even in infants and young children. The clinical manifestations of the disease are diversified and are not easily recognized, often leading to misdiagnosis.
Causes
The cause of nodular lipomatosis is still unclear, but it may be related to abnormal immune response and fat metabolism disorder.
Symptoms
1. Skin damage
Subcutaneous nodules are the main feature of this disease. Some of the nodules start from subcutaneous and develop upward, the skin surface may be mildly elevated, presenting erythema and small swelling; some of them are submerged in subcutaneous, and the surface skin is normal color, but they are often adherent to the skin, with small mobility, and obvious primary pain and tenderness. Nodules often occur in batches, symmetrical distribution, the most common site for the buttocks and lower limbs, but the lower anterior buttocks, trunk and face can also appear. Nodules recur every few weeks or months, with fever, fatigue, loss of appetite, muscle and joint pain and other manifestations.
2. Damage to internal organs
Liver damage may be characterized by right-sided dystocia, hepatomegaly, jaundice and abnormal liver function. Involvement of small intestine may have steatorrhea and intestinal perforation. Involvement of mesentery, greater omentum and retroperitoneal adipose tissue may cause epigastric pain, abdominal distension and masses. In addition, bone marrow, lung, pleura, myocardium, pericardium, spleen, kidney and adrenal glands can be involved.
Examination
1. Routine examination
The blood sedimentation rate increases significantly, and the white blood cell count is mildly elevated. If the liver and kidney are involved, there may be abnormal liver and kidney function, hematuria and proteinuria. Complement is decreased, immunoglobulin is increased and lymphocyte conversion rate is decreased. Bone marrow involvement may present with anemia, decreased white blood cells and low platelets.
2. Pathologic examination
In early lesions, there is a large number of inflammatory cells infiltration in adipose tissue, accompanied by focal fat necrosis. In the middle stage, there are histiocytic infiltration, granuloma formation and secondary vascular inflammatory changes. In the later stages, there is proliferation of fibroblasts in lobular intervals, formation of collagen, replacement of residual adipose tissue, and finally complete fibrosis.
Diagnosis
The diagnosis of nodular lipomatosis can be made on the basis of clinical manifestations combined with pathologic examination. The diagnosis is based on the following two points.
1. Clinical features: recurrent and mass occurrence of subcutaneous nodules with pain and tenderness; different degrees of depression and pigmentation after the nodules subside; often accompanied by fever, arthralgia and myalgia.
2. Pathological examination: the pathohistological changes of skin nodule biopsy is the main basis for diagnosis.
Treatment
There is no specific treatment for this disease.
1. General treatment: If infected lesions or allergens are found to exist, they should be removed in time.
2. Drug therapy: such as the use of corticosteroids, cytotoxic drugs, antimalarials, non-steroidal antipyretic and analgesic drugs.
Prognosis
The nodules of this disease recur every few weeks or months, and most attacks are accompanied by fever. Those with extensive visceral involvement may die from circulatory failure, hemorrhage, sepsis, and renal failure. In atopic restrictive seborrhea, the entire course of the disease lasts 6 to 12 months and eventually resolves.
Dietary management
1. High protein diet: such as oatmeal, pig heart, tofu skin, peanuts, pork (lean), etc.; high calorie diet: such as honey, sugar, animal and vegetable oils, etc.; high vitamin diet: such as cereals and fresh vegetables. These can replenish the nutrients lost by the skin in time.
2. Low-salt diet: low-salt diet means no more than 2 grams of salt per day.
3. Avoid eating seafood, chili peppers, raw onions, ginger and garlic, as well as alcohol, strong tea and coffee.