The clinical challenge of refractory eosinophilic fasciitis.

Abstract

To the Editor: Eosinophilic fasciitis (EF) is a rare connective tissue disease, with unclear etiology, characterized by hardening and thickening of the skin, mainly affecting the upper and lower extremities. This condition is associated with peripheral eosinophilia, elevated erythrocyte sedimentation rate (ESR), and hypergammaglobuli-nemia. Most patients with EF respond to high-dose corticosteroids. Thus, a case of EF is being reported for its rarity and partial response to prednisolone. We report a case of a 47-year-old woman with a personal history of multinodular goiter and no usual medication. This patient was admitted to the rheumatology service because of pain and skin hardening of right upper and lower limbs for the past 5 months. There were no systemic complaints; skin rash; Raynaud phenomenon; genital or oral ulcers; and respiratory, gastrointestinal, or genitourinary manifestations. No trauma or exacerbated physical activity was reported. During these months, the patient was medicated with an anti-inflammatory drug and low-dose corticosteroid for a small period, without significant relief. General physical examination was normal. On physical examination, skin thickening was observed on the right leg and forearm. On the forearm, the groove sign was visible when the patient raised the upper limb (Fig. 1). Left limbs, hands, and fingers were unaffected. There were no other mucocutaneous changes or peripheral arthritis. On investigation, she had peripheral eosinophilia (0.8 3 10 9 /L, normal range, , 0.5 3 10 9 /L), an elevated ESR (43 mm in the first hour), and proteinogram with a polyclonal hypergammaglobu-linemia