Eosinophilic fasciitis

Eosinophilic fasciitis or Shulman syndrome is a fibrotic disease of the fascia located. The etiology and pathophysiology are unclear.The disease is characterized by peripheral eosinophilia and necrotizing which can be differentiated from scleroderma skin by being distinctive avoid fingers, rather than involve the dermis and fascia is not accompanied by Raynaud's phenomenon.Fasceitei eosinophilic etiology remains unknown, although there have been suggesting different triggers and associated diseases.Some aspects of the pathology of the disease have been elucidated, but a complete understanding of the disease remains to be investigated.
Disease clinical picture includes critical elements: fascial thickening in eosinophilia, increased erythrocyte sedimentation rate and hypergammaglobulinemia. Impaired visceral eosinophilic fasceitei is generally absent, it helps to distinguish systemic sclerosis. Pair with some hematologic diseases is known and carries a negative prognosis.Eosinophilic fasciitis patient diagnosis is suspected in the presence of specific skin changes and laboratory studies. This is confirmed by biopsy and specific MRI imaging.
Eosinophilic fasciitis is responsive to corticosteroids and regimens are based on initial therapy. Numerous cases of necrotizing eoziofilica respond to corticosteroid therapy, a quarter of which get complete healing, although spontaneous resolution is possible. Full recovery may take 1-3 years. There is no consensus on treatment, but studies indicate that is the best treatment with moderate doses of corticosteroids, especially if started early in the disease.They used multiple additional agents in steroid refractory disease.Their outcome does not lead to a consensus on the best agent corticosteroid therapy for disease resistance. There are no data on mortality or morbidity disponivbile associated with the disease.Morbidity may be caused by contractions of joints or carpal tunnel syndrome associated with fascial fibrosis. Rarely can develop aplastic anemia.
Pathogenesis and causesAlthough the pathogenesis of the disease is unknown, studies have highlighted some of the mechanisms involved. Eosinophilic fasciitis generally involves an inflammatory response with inflammatory cell infiltration of affected tissue and impaired extracellular matrix secondary production by lesional fibroblasts.Fasciitis can be a common manifestation of various pathophysiological processes associated with eosinophilia. The existence of primary and secondary forms of fasciitis is a theory recently suggested.A few triggers include exercise and trauma. Various drugs are suspected: simvastatin, atorvastatin and phenytoin. Some cases were proven positive for infection with Borrelia. Serological significance of these findings is still debated.
Eosinophilic fasciitis shares clinical similarities with eosinophilia-myalgia syndrome. Some studies have suggested an association between intake of tryptophan and eosinophilic fasciitis. Despite these theories there is no consistent association between L-tryptophan and eosinophilic fasciitis.Hematologic diseases have been consistently reported. Their spectrum is broad and includes hemolytic and aplastic anemia, thrombocytopenia, myeloproliferative disease, myelodysplasia, lymphoma, leukemia and multiple myeloma.An association with thyroid disease was reported in several cases.Eosinophilic fasciitis has been rarely related to solid organ tumors and primary biliary cirrhosis. These associations suggest a common autoimmune pathophysiology.
Signs and symptomsThe disease is idiopathic, fibrotic fascial fibrosis. The presentation is an acute extremity pain, swelling, progressively debilitating cutaneous fibrosis. Joint contractures, arthritis, neuropathy and myositis may be associated with eosinophilic fasciitis. Many doctors believe the disease is a variant of morphea, and others a distinct entity.
Eosinophilic fasciitis affects white people more often than other breeds. In adults, affecting women more frequently than men. Age of onset of the disease is between 1-88 years, although many patients are presented in the fifth or sixth decade of life.
Patients with eosinophilic fasciitis shows symmetric swelling of the skin associated with tingling sensation in extremities affected, which may be fitted for an acute period of days or weeks. subacute disease may also occur. If the patient presents late in the disease, especially manifest symptoms of mercy and fibrosis of the affected areas.Onset of disease is accompanied by fever or other systemic symptoms. Up to half of patients the disease follows an episode of extreme exercise. Raynaud's phenomenon is not accompanying or symptoms of respiratory, gastrointestinal or cardiac. Inflammatory arthritis has been reported and is manifested by joint pain, swelling and morning stiffness. Once fibrosis patients may experience excessive motion by limiting joint contractures and paresthesia distribution similar to carpal tunnel syndrome.
Physical Exam:Skin manifestations:Skin manifestations of eosinophilic fasceitei evolve as the disease progresses. The acute stage inflammatory changes include skin redness, and swelling without pitting. These elements are later replaced by mercy skin fibrosis and eventually prevailed. Affected skin is firmly adherent to the underlying tissue. Other skin manifestations include urticaria, blistering, alopecia, lichen sclera and atrophy, vitiligo and hyperpigmentation. Skin manifestations are usually bilateral and symmetrical. Upper extremity, proximal and distal to the elbow and lower extremity, proximal and distal to the knee are the most involved areas. Trunk and neck may also be affected. Face and hands are rarely involved. Localized morphea may be observed in 25% of patients.
Extracutaneous manifestations:Joint contractures are the most common extracutaneous manifestation of eosinophilic fasceitei. Occurs in 75% of patients and affects the elbow, wrists, knees, ankles and shoulders.Truncata extensive fibrosis may limit chest expansion.Inflammatory arthritis is reported in 40% of patients. Knee, wrist, hands and feet seem to be most involved. Carpal tunnel syndrome is seen in 23% of patients.Clinically significant visceral involvement is rare. If this requires suspicion of another diagnosis. Pulmonary tests, esofagogastroduodenoscopia and electromyography can demonstrate subtle or nonspecific abnormalities.
Disease progression:The final stage of fibrotic process leads to substantial morbidity with skin sclerosis and joint contractures. Arthritis, neuropathy and myositis may be present. 10% of cases may cause myelodysplasia: aplastic anemia with negative prognosis.Spontaneous resolution is possible and treatment with corticosteroids usually result in recovery, skin fibrosis and contractures may still persist.