The patient was prescribed prednisolone at 30 mg/day and methotrexate at 20 mg/week, with moderate clinical and radiological improvement, and complete recovery of joint mobility. Systemic corticosteroids were gradually tapered… Click to show full abstract
The patient was prescribed prednisolone at 30 mg/day and methotrexate at 20 mg/week, with moderate clinical and radiological improvement, and complete recovery of joint mobility. Systemic corticosteroids were gradually tapered to 5 mg/day and methotrexate dosage was maintained with good tolerance. Eosinophilic fasciitis (Shulman’s syndrome) is a rare fibrosing disorder of unknown aetiology, which is likely to be underdiagnosed. It is characterized by an acute onset of symmetrical and painful cutaneous oedema and induration of the four extremities, accompanied by peripheral eosinophilia. The hands and feet are normally spared. Areas of orange peel-like or “pseudo-cellulite” appearance and linear depressions along the course of the superficial veins (“groove sign”) are typical. Joint contractures may be present and are responsible for the high morbidity. A full-thickness incisional biopsy including the fascia and/or magnetic resonance are crucial for diagnosis. The presence of tissue eosinophilia is not necessary for the diagnosis. Systemic corticosteroids are the first-line treatment. Methotrexate may be used in combination as a corticosteroid-sparing agent [1, 2].
               
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