Spontaneous panniculitis: causes, symptoms, diagnosis, treatment

Panniculitis spontaneous (synonyms: Weber-Christian disease, atrophic hypodermis).

The causes and pathogenesis of spontaneous is not fully understood. Of great importance are the transferred infections, trauma, drug intolerance, pancreatic lesions, etc. A certain role is played by the processes of lipid peroxidation. The intensification of these processes in the pathological focus and decrease in antioxidant activity of the organism was revealed. There is a deficit of a-antitrypcilin (a-ANT) - an inhibitor of plasma proteases. A-ANT controls the development of inflammatory reactions.

Symptoms of panniculitis spontaneous. The disease is more common in women. It usually begins with malaise, pain in the muscles and joints, headache, increase in body temperature to 37-40 ° C. Sometimes the disease begins and proceeds without disturbing the general condition of the patient. The appearance of single ears of multiple subcutaneous nodes is characteristic. The process can be disseminated. The nodes merge into conglomerates of various sizes, forming on the surface extensive plaques with an uneven, bumpy surface and diffuse boundaries. In the classical course, nodes are usually not opened, with resorption in their place remain areas of atrophy or occlusion. Sometimes on the surface of individual nodes or conglomerates there is a fluctuation, the nodes are opened, releasing a yellowish foamy mass. The skin above the nodes has a normal color or gets a bright pink color. Most nodes are located on the lower and upper limbs, buttocks, but can be localized in other areas.

Clinically, there are three forms of skin manifestations of spontaneous panniculitis: nodular, plaque and infiliprative.

Knot form is characterized by the formation of nodes ranging in diameter from a few millimeters to several centimeters. Depending on the depth of the subcutaneous tissue they have a bright pink or pink-cyanotic color, are isolated from each other, do not merge, are clearly delineated from the surrounding tissue.

The plaque form is formed as a result of the fusion of nodes into conglomerates that occupy the vast surfaces of the shin, thigh, shoulder, etc. In this case, edema of the limb and severe pain can be noted due to compression of the neurovascular bundles. The surface of the lesions is tuberous, the boundaries are vague, the consistence is dense-elastic (scleroderm-like). The color of the skin in the lesions varies from pink to bluish-brown.

When infiltrative form on the surface of individual nodes or conglomerates appears fluctuation, the color of the foci becomes bright red or crimson. The clinical picture resembles an abscess or phlegmon. When the foci are opened, a yellowish foamy mass is detected.

One patient may have different forms at the same time, or one form may change to another.

Depending on the severity of the clinical course, chronic, subacute and acute forms are isolated.

In acute course, there are marked general symptoms of the disease: long-term hectoric fever, weakness, leukopenia, increased ESR; deviation of biochemical constants of the organism. Clinically, this form is characterized by rapid change of remissions and relapses, torpidity and resistance to various types of therapy. The number of nodes on the skin is usually increased. In subacute form, clinical signs are weaker.

The chronic form of spontaneous panniculitis has a favorable course, the general condition of patients is usually not disturbed, remissions last for a long time, but relapses are difficult. Changes in internal organs are absent.

Histopathology. Histological changes are characterized by infiltration of fatty tissue with lymphocytes, segmented neutrophils, without the formation of abscesses. As the pathological process develops, the infiltrate, consisting of histiocytes with an admixture of plasma cells and lymphocytes, replaces whole fat segments. There are histiocytes that absorb fat, which has come out of dead fat cells, and the remains of the fat cells themselves - the so-called foam cells. In the final stage, the process ends with an increase in the number of fibroblasts and the replacement of foci with a connective tissue.

Differential diagnosis. The disease should be differentiated from post-steroids panniculitis, insulin lipodystrophy, oleogranuloma, erythema nodosum, deep subcutaneous sarcoid, deep form of lupus erythematosus, lime.

Treatment of panniculitis spontaneous. The treatment takes into account the general condition of the patient, the clinical variety of spontaneous panniculitis and the nature of the course of the disease. Assign antioxidants (alpha-tocopherol, lipid, lipoic acid), fortifying agents (large doses of ascorbic acid, rutin), broad-spectrum antibiotics, antimalarial drugs. In severe and persistent flow, systemic corticosteroids (prednisolone 50-100 mg), cystostics (prospidin) are effective. Outwardly the foci is lubricated with 5% liniment of dibunol 2-3 times a day, under the occlusive dressing - once a day.

[1], [2], [3], [4], [5], [6]