Dapsone Treatment for Eosinophilic Fasciitis

Abstract

A previously healthy 38-year-old woman was seen regarding severe skin stiffness and thickening. Previous findings are summarized. The patient originally saw her primary care practitioner with a 3-month history of limb aches, ankle swelling, and malaise. She was taking no medication. Findings showed an increased serum C-reactive protein level (80 mg/L [to convert to nanomoles per liter, multiply by 9.524]), mild anemia (hemoglobin level, 11.3 g/dL [to convert to grams per liter, multiply by 10.0]), elevated plasma viscosity (1.74 mPa·s), eosinophilia (eosinophil count, 2.92 10/μL[toconvert tovalue 10/L,multiplyby10]), and low serum ferritin and folate levels. A gastroenterologic consultation to investigate suspected malabsorption excluded celiac disease, parasitic infection of the bowel, and a gastrointestinal or gynecological malignant neoplasm. Computed tomography of the abdomen showed only a thickened proximal jejunal loop. Because of persisting eosinophilia (eosinophil count, 4.77 10/μL and 6.97 10/μL on 2 further occasions), hypereosinophilic syndrome was considered. The jejunal abnormalities were presumed to represent eosinophilic enteritis, but this condition was excluded histologically. Because of increasing symmetrical arthralgias, the patient was seen in the rheumatology department. Generalized tightening and tautness of the skin, restricted joint movement (particularly the elbow and metacarpophalangeal [MCP] joints), and a positive prayer sign (inability to oppose the palms of the hands) were documented. A negative result for rheumatoid factor and normal autoimmune screening findings and complement levels made scleroderma spectrum disorders unlikely. A polyclonal increase in -globulins (mainly IgG) was found. In the dermatology department, the stiff induration and predominantly distal limb distribution of skin tethering, together with significant blood eosinophilia, were viewed as highly suggestive of eosinophilic fasciitis (EF). A deep incisional biopsy to the fascia, from the right forearm, supported this diagnosis. Septae between fat lobules were thickened by fibrosis, and a mononuclear infiltrate including numerous eosinophils was seen. Overlying skin appeared normal. Treatment with oral prednisolone was started, initially at 40 mg/d. After 2 weeks, some symptomatic improvement and a reduction of skin edema were apparent, so the prednisolone dose was reduced to 30 mg/d. At review some weeks later, however, there had been little, if any, further improvement.