Eosinophilic fasciitis

Eosinophilic fasciitis is a rare disease characterized by symmetrical and painful inflammation, swelling and induration of the skin of the lower and upper extremities.

Diagnosis is established by skin and fascia biopsy data. Treatment is reduced to the use of glucocorticoids.

[1], [2]

Causes of the eosinophilic fasciitis

The causes of eosinophilic fasciitis are unknown. The most common are middle-aged men, but eosinophilic fasciitis can also occur in women and children.

[3], [4], [5], [6], [7], [8], [9]

Symptoms of the eosinophilic fasciitis

The onset of the disease is often observed in people leading a sedentary lifestyle, after performing heavy physical work (for example, after splitting firewood). The pain, swelling and inflammation of the skin and subcutaneous tissues develop, followed by their densification, which leads to a characteristic skin change, like the "orange peel", which is most pronounced on the forelimb surfaces. The skin of the face and body are less likely to be affected. Following the induction and thickening of the fascia, the limitation of the volume of movements in the joints of the upper and lower extremities develops; In addition, the process may involve tendons, synovial vaginas and muscles. The defeat of the fingers and feet is not typical for eosinophilic fasciitis. The strength of the muscles usually does not suffer, but the development of arthritis and myalgia, as well as carpal tunnel syndrome, is possible.

Typical fatigue and weight loss. Often develop aplastic anemia, thrombocytopenia and lymphadenopathy.

[10], [11], [12], [13], [14]

Diagnostics of the eosinophilic fasciitis

Eosinophilic fasciitis should be suspected if the patient has typical manifestations. Skin changes should be differentiated with those in systemic scleroderma; but it is usually characterized by the phenomenon of Reynaud, damage to the distal parts of the limbs, the appearance of telangiectasias and disorders of the internal organs (for example, atony of the esophagus), which is not observed with eosinophilic fasciitis.

Diagnosis is established by microscopy of biopsies of the altered skin and fascia, and the biopsy material must contain muscle fibers. In favor of the diagnosis, the presence of inflammation of the fascia with or without eosinophils is indicated.

Blood tests are usually of little informative, however, in general blood analysis, eosinophilia can be detected (especially in the active initial phase of the disease), polyclonal hypergammaglobulinemia is detected by electrophoresis of blood proteins. Autoantibodies are usually not detected. The results of MRI, although not specific, allow us to establish the presence of thickening of the fascia, accompanied by an increase in the intensity of the signal of the superficial muscles, which correlates with the severity of inflammation.

[15], [16], [17], [18], [19], [20], [21], [22]

Treatment of the eosinophilic fasciitis

Most patients respond quickly to treatment with high doses of prednisolone (orally, 40-60 mg once a day, followed by a decrease to 5-10 mg / day as the disease progresses). Accepting low doses of glucocorticoids can last for 2 to 5 years. Despite the different outcomes of the disease, eosinophilic fasciitis is often stopped independently, without the development of complications. However, due to the possibility of developing hematological disorders, it is recommended to monitor the parameters of a clinical blood test.