What is Diffuse (Eosinophilic) Fasciitis?
Diffuse (eosinophilic) fasciitis D (E) F is a systemic disease of the connective tissue with a primary lesion of the deep fascia (inflammation with an outcome in fibrosis), subcutaneous tissue and underlying muscles and dermis, accompanied by eosinophilia and hypergamma globulinemia. An ESP was isolated in 1975 by L. E. Shulman from systemic scleroderma as a scleroder syndrome like syndrome.
- Epidemiology
D (E) F has not been studied due to the relative rarity of the disease. The spread of the disease is evident, since according to the literature there are more than 100 observations of patients from various countries of the world. In our country, the first description of EF refers to 1978, a total of 15 cases are described. Among the patients with EF, middle-aged men predominate, but children and the elderly are ill.
- Pathomorphology
Studied quite well through the study of skin biopsy, underlying tissues and muscles. The epidermis and the upper layers of the dermis are usually normal, sometimes thickened, swollen. In the deep layers of the dermis and in the subcutaneous tissue, perivascular histiocytic infiltrates are found along the connective tissue layers, with the inclusion of individual eosinophils and the development of fibrosis. Especially significant changes are found in the fascia – it is thickened many times in comparison with the norm, infiltrated with a large number of lymphocytes and histiocytes with an admixture of plasma cells and eosinophils. In the fascia, fibrinoid changes in collagen fibers are determined up to fibrinoid necrosis. Subsequently in the fascia develops fibrosis. Connective tissue layers with pronounced signs of inflammatory infiltration; sometimes there are signs of damage to the muscle fibers. In the late stages of the disease, with the predominance of the processes of sclerosis in the affected tissues, the differential diagnosis with SJS is difficult.
Causes of Diffuse (Eosinophilic) Fasciitis
The etiology and pathogenesis of the disease are not well understood. Excess cooling, physical overstrain, for example, carrying weights, sports overtraining, and injuries are involved as provoking factors. Less commonly, D (E) F develops after an acute infection or an allergic reaction. Perhaps the role of genetic predisposition. For example, M. Thibierge notes cases of focal scleroderma, RA, and diabetes in the families of patients.
Among the developmental mechanisms of D (E) F, considerable attention is paid to immune disorders, which is manifested by hypergamma globulinemia and an increase in the content of immunoglobulins, especially IgG, circulating immune complexes and the development of inherently inflammatory inflammation in the deep fascia and surrounding tissues. S. J. Wasserman et al. They proposed an original hypothesis of the pathogenesis of ESP, according to which the initial link is antigenic alteration of the most affected tissues, including muscles, as a result of excessive exercise or other causes, with eosinophils entering the lesion, possibly due to lymphokine and immunocomplex or specific eosinophilic activity. Phagocytes attracted to the affected tissues stimulate the development of inflammation and fibrosis, which are the main clinical manifestations of the disease. Also of interest is the concept of G. Solomon et al., Which is based on the predominant disruption of cellular immunity, the involvement of a subpopulation of Thymphocytes possessing H2 receptors. The authors obtained a satisfactory effect from the use of cimetidine, which is an H2 receptor antagonist. Consequently, the issues of the etiology of the pathogenesis of EF have not yet been resolved, however, the peculiarity of the disease is excessive eosinophilic activity, which is determined in the majority of patients, as well as dysfunction of Tsupres sor. Various interrelations of the main pathogenetic processes determine the clinical features of the course of D (E) F.
Symptoms of Diffuse (Eosinophilic) Fasciitis
The clinical picture is characterized by compaction of the soft tissues of the upper and lower extremities with disruption of their motor activity, up to the development of flexion contractures in various joints, mainly fingers.
One of the earliest signs of the disease is the appearance of a feeling of tightening of the skin in the region of the upper and (or) lower extremities, a sensation of swelling and density, less often an itch. Almost simultaneously, there is a limitation of movement in the hands, weakness in the legs when climbing the stairs. Characteristic is the development of compaction of the soft tissues of the forearms and / or shins, extending to the shoulders and hips, appearing over several months or even days. It is extremely rare for moderate compaction to be found on the neck, face, abdomen, or on the body. Seals are usually non-painful, although occasionally patients complain of moderate spontaneous pain in these areas.
The structure of the skin is usually not changed, or in places of compaction it becomes taut, shiny, and occasionally hyperpigmented with signs of hyperkeratosis. Characterized by the “orange peel” symptom in the form of soft contractions with maximum tension, i.e. maximum extension of the limb (on the inner surface of the shoulders, thighs). In all patients, there is a restriction of active movements in the joints of the extremities due to their consolidations, up to the development of stable contractures mainly in the fingers, less often in the elbow and knee joints.
The joints are not changed, arthritis is not detected, but in some patients polyarthralgia can be observed as a manifestation of the overall activity of the disease. Much more often, patients complain of polymyalgia and muscle weakness, although clinically no signs of myositis are found.
Lesions of internal organs, vascular disorders, including Raynaud’s syndrome, no trophic abnormalities are noted, which distinguishes EF from SJS. There may be unstable subfebrile.
Among laboratory parameters, eosinophilia up to 10-44%, accompanied by hypergamous maglobulinemia, has diagnostic value. In 50% of patients, an ESR increased to 20–30 mm / h, less frequently to 50 mm / h, an increase in CRP is noted. In the acute stage of the disease, the content of seromucoid, fibrinogen, ceruloplasmin is increased in patients, but only slightly.
Changes in humoral immunity are uncharacteristic. More often increases the number of IgG, less CEC are detected and extremely rarely ANF.
Diagnosis of Diffuse (Eosinophilic) Fasciitis
Diagnosis D (E) F is difficult when detecting characteristic seals on the upper and lower extremities, the symptom of orange peel, restriction of movement, up to the development of flexion contractures. Hypereosinophilia and hypergammaglobulinemia are of diagnostic value.
The biopsy of the entire complex of tissues affected by D (E) F of the skin, subcutaneous tissue, fascia, muscle has a diagnostic value. At the same time, pathological changes in the fascia are characteristic – a significant thickening with signs of inflammation and fibrosis.
However, due to lack of qualification, doctors often mistakenly make the wrong diagnosis (SJS, dermatosis or fibromyositis, tendovaginitis, RA, etc.). According to N. G. Guseva et al. , only 2 out of 10 patients were referred to the clinic with the correct diagnosis of D (E) F. Thus, first of all, D (E) F should be differentiated from SJS and scleroderm-like syndromes.
SSD differs from D (E) F in the presence of Raynaud’s syndrome, dense swelling of the fingers and hands, dilatation of the esophagus, pulmonary lesions like basal pulmonary fibrosis and the development of other viscerites. At the same time, it is necessary to bear in mind the possibility of combining SSD and D (E) F.
Buske scleroderma differs from D (E) F in the development of dense edema of the upper torso and proximal extremities, the lack of morphological changes in the fascia area typical of EF in the affected area.
The absence of myositis, dermatitis and arthritis makes it possible to distinguish D (E) F from dermatomyositis and RA. For tendovaginitis, a local asymmetrical lesion of one or another tendon is characteristic, while with an EF process it is always symmetrical with its inherent external changes in tissues and eosinophia.
The presence of a dense symmetrical edema and a characteristic bioptate allows to distinguish EF from hypereosinophilia of different origin.
Treatment of Diffuse (Eosinophilic) Fasciitis
Drugs are effective in the early period of the disease, but often the gradual development of the process leads to untimely recognition and late treatment. As with other diseases from the DBST group, EF requires long-term, often long-term, complex treatment.
Since the signs of inflammatory activity appear in the first place in the clinical picture of the disease, the GCS is most indicated, mainly prednisone at 20-30 mg/day, more rarely (60-70 mg/day). In suppressive doses, prednisone is prescribed to reduce the activity of the process, reduce the subjective and objective signs of the disease. In the future, the dose of prednisone is gradually reduced to supporting, which often must be prescribed for many months and even years (up to 8 years, according to NG Guseva et al.)
NSAID therapy is usually ineffective.
With the ineffectiveness of GCS treatment, cytostatics are additionally prescribed, mainly azathioprine 150 mg/day for several months, and with fibrosis, Dpeni Cillamine up to 450-600 mg/day. The duration of medication is determined by the presence of inflammatory, immune or fibrotic changes.
As a local anti-inflammatory and anti-fibrosing therapy, the treatment complex includes courses of DMSO applications (50%), trilon B phonophoresis on the affected areas. With a decrease in the activity of the process are exercise therapy, massage courses.
In case of torpid course of the disease and ineffectiveness of therapy, according to N. G. Guseva et al., Hemo sorption is recommended. It is possible that extracorporeal therapy is indicated in the earlier stages of the disease due to increased activity of eosinophilic factor.
According to S. Herson et al., In the early stages of the disease, prednisone should be prescribed at 0.35-0.6 mg/kg in combination with cimetidine at a dose of up to 1000 mg/day as an H2 receptor blocker.
Thus, the effectiveness of treatment depends on the timing of its start. With early recognition and systematic treatment, almost all patients by the end of the 1st year develop significant improvement or clinical remission. However, with late recognition and the development of fibrosis, the treatment results are much worse.
Prevention of Diffuse (Eosinophilic) Fasciitis
Primary prevention is not developed. Secondary prevention of exacerbation is associated with the systematic treatment of complex therapy.