Eosinophilic Fasciitis: A Difficult Diagnosis

Eosinophilic fasciitis (EF) often is confused with scleroderma. A 50-year-old woman presented with progressive tightening of the skin of the arms and legs. She complained of diffuse joint pain that was prominent in the hands and wrists. Her past medical history was significant for hypothyroidism. A skin biopsy revealed superficial and deep infiltrates of inflammatory cells with eosinophils. Physical examination findings were significant for skin hypopigmentation and hyperpigmentation over her forearms and legs. EF may be difficult to differentiate from localized scleroderma, limited and diffuse cutaneous systemic sclerosis, and several scleroderma-like disorders. (J Musculoskel Med. 2012;29:45-47, 55) Eosinophilic fasciitis (EF), also known as Shulman disease and diffuse fasciitis with eosinophilia, is an uncommon condition that often is confused with scleroderma because they have similar features. Shulman1 first described EF in 1974 when he saw patients with scleroderma-like skin changes but histopathological features on deep biopsy showed evidence of diffuse fasciitis. In this article, we describe an unusual case of a patient who presented to our academic clinic for consultation with either scleroderma or severe, advanced morphea. CASE PRESENTATION A 50-year-old white woman presented to our clinic with progressive tightening of the skin of the arms and legs that had started 8 months earlier when she was packing with her family to move to Florida. She stated that the packing took too many long hours and was very strenuous. She had noticed a hardened patch of skin on her left lower leg above the ankle, but she did not recall experiencing any injury to that area while she was packing. Progressive hardening of her arms and legs with hypopigmented and hyperpigmented skin developed gradually over a few months but spared her hands and feet. She complained of joint pain that was diffuse but was prominent in the hands and wrists, which appeared swollen and puffy. She denied any history of Raynaud phenomenon (RP), shortness of breath, chest pain, fatigue, or dysphasia. The patient started to receive 10 mg/d of prednisone a few months later, and her joint pain, swelling, and arthritis resolved completely. However, the skin tightness continued to progress to involve the whole arms and legs while sparing the trunk, face, hands, and feet. Some areas of the skin on her upper arms and thighs appeared “to have hardened wrinkles or to be cellulite-like in the thigh area,” as the patient described it. Past Medical History The patient’s past medical history was significant for hypothyroidism. That diagnosis had been made 7 years earlier, when she was working as a respiratory therapist at a hospital in Virginia and was complaining of progressive fatigue. She denied having had any similar skin lesions in the past. However, 7 years earlier she had had one area of erythema and slightly hardened skin over her right ankle. It was itchy but resolved in a few months without complications or scarring. Her treating physician told her at the time that she had morphea. The patient is married and has 6 children. She denied any history of drug, alcohol, or tobacco use, and her family history was negative for any autoimmune connective-tissue disease. Otherwise, findings from a review of systems were completely unremarkable. She was taking only the prednisone at 10 mg/d, her thyroid replacement medication, a multivitamin, and fexofenadine as needed for chronic seasonal allergies. A skin biopsy specimen was obtained from the patient’s right forearm at the time when her joint symptoms had resolved. It revealed superficial and deep infiltrates of inflammatory cells with eosinophils. Blood work done a month earlier showed a negative antinuclear antibodies (ANAs) test result and normal thyroid-stimulating hormone and creatine phosphokinase levels. Tests for