Spontaneous panniculitis: causes, symptoms, diagnosis, treatment

Spontaneous panniculitis (synonyms: Weber-Christian disease, atrophic hypodermitis).

The causes and pathogenesis of spontaneous panniculitis have not been fully studied. Past infections, trauma, drug intolerance, pancreatic lesions, etc. are of great importance. Lipid peroxidation processes play a certain role. An increase in these processes in the pathological focus and a decrease in the antioxidant activity of the body have been revealed. A deficiency of a-antitrypticlin (a-ANT), an inhibitor of plasma proteases, is noted. a-ANT controls the development of inflammatory reactions.

Symptoms of spontaneous panniculitis. The disease is more common in women. It usually begins with malaise, muscle and joint pain, headache, and an increase in body temperature to 37-40 °C. Sometimes the disease begins and progresses without disturbing the general condition of the patient. The appearance of single or multiple subcutaneous nodes is characteristic. The process may be disseminated. The nodes merge into conglomerates of various sizes, forming extensive plaques on the surface with an uneven, bumpy surface and blurred boundaries. With a classic course, the nodes usually do not open; when they resolve, areas of atrophy or depression remain in their place. Sometimes fluctuation appears on the surface of individual nodes or conglomerates, the nodes open, releasing a yellowish foamy mass. The skin over the nodes has a normal color or becomes bright pink. Most often, the nodes are located on the lower and upper limbs, buttocks, but can also be localized in other areas.

Clinically, three forms of cutaneous manifestations of spontaneous panniculitis are distinguished: nodular, plaque and infiltrative.

The nodular form is characterized by the formation of nodes with a diameter from several millimeters to several centimeters. Depending on the depth of their location in the subcutaneous tissue, they have a bright pink or pink-blue color, are located isolated from each other, do not merge, and are clearly delimited from the surrounding tissue.

The plaque form is formed as a result of the fusion of nodes into conglomerates that occupy large areas of the shin, thigh, shoulder, etc. In this case, swelling of the limb and severe pain due to compression of the vascular-nerve bundles may be observed. The surface of the lesions is bumpy, the boundaries are blurred, the consistency is densely elastic (scleroderma-like). The skin color in the lesions varies from pink to bluish-brown.

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

One patient may experience different forms at the same time, or one form may develop into another.

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

In the acute course, pronounced general symptoms of the disease are observed: prolonged hectic fever, weakness, leukopenia, increased ESR; deviation of the body's biochemical constants. Clinically, this form is characterized by a rapid change of remissions and relapses, torpidity and resistance to various types of therapy. The number of nodes on the skin usually increases. In the subacute form, clinical signs are less pronounced.

The chronic form of spontaneous panniculitis has a favorable course, the general condition of patients is usually not disturbed, remissions last a long time, but relapses are severe. There are no changes in the internal organs.

Histopathology. Histological changes are characterized by infiltration of adipose tissue by 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 entire fat lobules. Histiocytes appear, absorbing fat released from 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 connective tissue.

Differential diagnosis. The disease should be differentiated from poststeroid panniculitis, insulin lipodystrophy, oleogranuloma, erythema nodosum, deep subcutaneous sarcoid, deep lupus erythematosus, lipomas.

Treatment of spontaneous panniculitis. The treatment takes into account the general condition of the patient, the clinical type of spontaneous panniculitis and the nature of the disease. Antioxidants (alpha-tocopherol, lipamide, lipoic acid), tonics (large doses of ascorbic acid, rutin), broad-spectrum antibiotics, antimalarial drugs are prescribed. In severe and persistent cases, systemic corticosteroids (prednisolone 50-100 mg), cygostatics (prospidin) are effective. Externally, the lesions are lubricated with 5% dibunol liniment 2-3 times a day, under an occlusive dressing - once a day.

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