Stages of Eosinophilic Fasciitis

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What are the three stages of Eosinophilic Fasciitis?

• Stage I: pitting and edema. Disease onset is commonly acute, with simultaneous involvement of affected areas and rapid spread of skin disease. The most frequent pattern of involvement includes bilateral arms and legs (most commonly the calves and forearms) in a symmetrical fashion with sparing of the fingers and toes. Raynaud’s phenomenon has rarely been reported, but nailfold capillaroscopy is normal.

• Stage II: the initial manifestations of EF are followed by the development of severe induration of the skin and subcutaneous tissues of affected sites. These histologic changes manifest clinically as a “woody”, firm texture of involved skin, with a coarse orange-peel appearance (peau d’orange) . The groove sign is an indentation caused by tethering of the dermis to the fascial and muscular tissue layers 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. Inflammatory and fibrotic disease extends to the muscle and can result in a low-grade myositis, although creatine phosphokinase (CPK) levels are commonly normal.

• Stage III: because of the 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.

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