Eosinophilic fasciitis is a diffuse connective tissue disease characterized by lesions of deep fascia, muscles, subcutaneous tissue, skin and occurring against the background of eosinophilia and hypergammaglobulinemia. For the clinical picture of eosinophilic fasciitis, the skin is typically compacted by the type of “orange peel”, edema of the distal extremities, polyarthralgia, flexion contractures of the joints, myalgia. When diagnosing Eosinophilic fasciitis, clinical symptoms, laboratory data (hypereosinophilia, hypergammaglobulinemia), skin and muscle biopsy results are taken into account. Therapy includes the appointment of corticosteroids, NSAIDs, cytostatics, extracorporeal hemocorrection.
Eosinophilic fasciitis is a disease of the collagenosis group that occurs with primary inflammatory fascia damage and subsequent scleroderm–like changes in the dermis, subcutaneous base and muscles. The disease was named after the American rheumatologist L.E. Schulman, who in 1974 isolated eosinophilic fasciitis into an independent nosological form from the group of systemic scleroderma. Eosinophilic fasciitis belongs to the category of rare: about 150 such observations have been described in world medicine, and only isolated cases in domestic rheumatology. Disease develops mainly at the age of 25-60 years; men are 2 times more likely than women.
The etiology and risk factors have not been sufficiently studied. It is known that the onset of the disease is often preceded by excessive physical exertion (lifting weights, intensive sports training), operations, injuries, allergic reactions, acute infections, hypothermia, neuropsychiatric overstrain. The role of genetic predisposition is being studied: some authors point to the development of eosinophilic fasciitis in patients whose relatives suffered from focal scleroderma, diabetes mellitus, rheumatoid arthritis. There are indications of the connection with diseases of the blood system (thrombocytopenia, chronic myeloid leukemia and lymphocytic leukemia, lymphogranulomatosis), as well as Sjogren’s disease, polymyositis, autoimmune thyroiditis.
The mechanism of development of diffuse eosinophilic fasciitis is associated with immune disorders, which is confirmed by hypergammaglobulinemia, CEC, an increase in the level of immunoglobulins, mainly IgG, as well as the autoimmune nature of inflammation of deep fascia and nearby tissues. The morphological basis is the formation of inflammatory infiltrates containing a large number of eosinophils, and their subsequent transformation into fibrosis. Despite the similarity of eosinophilic fasciitis with systemic scleroderma, patients with eosinophilic fasciitis do not have Raynaud’s syndrome and visceral pathology.
Diffuse eosinophilic fasciitis usually manifests acutely or subacutely a few days after exposure to provoking factors. At the beginning of the disease, nonspecific general symptoms are noted: malaise, subfebrility, myalgia and arthralgia. Against this background, a dense painful “pillow-like” swelling of the skin of the distal extremities – feet, shins, hands, forearms – gradually or suddenly develops. Less often, the swelling spreads to the face, neck, fingers, hips, trunk. Foci can be located symmetrically or asymmetrically, be single or multiple. In places of seals, the skin is stretched and shiny; when the limbs are extended and the skin is stretched to the maximum, the symptom of “orange peel” occurs.
Subjectively, patients with eosinophilic fasciitis feel burning, itching, swelling, tightening of the skin. Focal or diffuse hyperpigmentation, hyperkeratosis occurs in the affected areas. Since the inflammatory process spreads to tendons, muscles, synovial membranes of joints, clinical manifestations are supplemented by flexion contractures of the fingers, knee, elbow and shoulder joints; oligo- or polyarthritis, carpal tunnel syndrome. All this leads to limited mobility of the limbs, difficulties when walking, climbing stairs, raising hands, etc.
The difficulties of timely recognition are primarily associated with the rare occurrence of this pathology in clinical practice. However, all patients with suspected diffuse eosinophilic fasciitis should be referred to a rheumatologist to verify the diagnosis. In order to exclude skin diseases in the initial stage, it may be necessary to consult a dermatologist.
Along with clinical signs, laboratory indicators play an important role in confirming eosinophilic fasciitis, namely high eosinophilia, hypera2- and γ-globulinemia, an increase in ESR, fibrinogen, seromucoid, ceruloplasmin, IgG and IdM, the presence of CRP, CEC, etc. In isolated cases, positive antinuclear antibodies, ANF and rheumatoid factor are noted.
The results of a biopsy of the skin, muscles and muscle fascia are crucial in the diagnosis of eosinophilic fasciitis. Morphological changes in tissues are characterized by thickening and swelling of the fascia, perivascular accumulation of lymphocytes, histiocytes and eosinophils; in the later stages – the predominance of sclerosis and hyalinosis. Differential diagnosis is carried out with dermatomyositis, systemic scleroderma, Buschke scleredema, fibromyositis, tendovaginitis, rheumatoid arthritis, allergic dermatitis, etc.
Treatment and prognosis
Therapy of eosinophilic fasciitis is most effective in early detection of the disease; at the same time, treatment should be long-term, systematic and comprehensive. In order to stop inflammatory activity, glucocorticosteroids (prednisone) are prescribed, sometimes in combination with NSAIDs, H-receptor blockers (cimetidine). Additionally, treatment with cytostatics (azathioprine) may be required, and with the development of fibrosis – D-penicillamine. With a persistent and severe course of eosinophilic fasciitis, hemosorption is used. Local anti-inflammatory and antifibrosing therapy includes applications of dimexide, ultraphonophoresis of the disodium salt of ethylenediaminetetraacetic acid. Outside of the exacerbation of eosinophilic fasciitis, massage and physical therapy courses, diathermy, warm therapeutic baths are added.
As a result of timely initiated and pathogenetically justified therapy, by the end of the first year of treatment, most patients have an improvement in their condition or achieve clinical remission. The literature describes cases of spontaneous regression of pathology. However, there are also less favorable variants of diffuse eosinophilic fasciitis with a persistent course and the development of persistent residual contractures.