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Eosinophilic Fasciitis (Diffuse Fasciitis with Eosinophilia - Shulman Syndrome)

Eosinophilic fasciitis is a rare scleroderma-like disorder characterised by inflammation, swelling and thickening of the skin and fascia.

Eosinophilia is often present only during the early stages of a patient’s illness and tends to decline later in the illness. The degree of eosinophilia does not closely parallel disease activity and resolves quickly with corticosteroid therapy.

Stages of eosinophilic fasciitis:

Stage I: pitting and edema. The most frequent pattern includes involvement of both the arms and legs in a symmetrical fashion with sparing of the fingers and toes. The proximal areas of the extremities are generally more affected than the distal. Raynaud’s does not occur.

Stage II: the initial manifestations of eosinophilic fasciitis (EF) are often followed by the development of severe induration of the skin and subcutaneous tissues of the affected areas. The skin becomes taut and woody with a coarse orange-peel appearance (peau d’orange). The groove sign is an indentation caused by retraction of the subcutaneous tissues along the tract of superficial veins and is best seen with elevation of the extremity. Although the induration often remains confined to the extremities, it may variably affect extensive areas of the trunk and face. A low-grade myositis with normal creatine phosphokinase (CPK) can occur.

Stage III: because of involvement of the fascia, carpal tunnel syndrome is an early feature in many patients. Flexion contractures of the digits and extremities may occur as a consequence of the fascial involvement. Muscle atrophy can be prominent. Sclerodactyly and nailfold capillary abnormalities do not occur.

If untreated, fascial inflammation will lead to joint contractures in 85% of patients. In addition, the skin that is initially indurated frequently may become bound down and develop a peau d’orange appearance. In some patients, the illness is self-limited with spontaneous improvement. Complete remission can occur even after 2 or more years. Young age at onset and trunk involvement are poor prognostic signs.





Rare Diseases (NORD)




Rheumanetz Österreich (German)

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