Eosinophilic fasciitis: an atypical presentation of a rare disease

Abstract

SUMMARY Eosinophilic fasciitis, or Shulman's disease, is a rare disease of unknown etiology. It is characterized by peripheral eosinophilia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. The diagnosis is confirmed by a deep biopsy of the skin. The first line of treatment is corticotherapy. We present a rare case of eosinophilic fasciitis in a 27-year-old woman with an atypical presentation with symmetrical peripheral edema and a Groove sign. The patient responded well to treatment with corticosteroids at high doses and, in this context, was associated with hydroxychloroquine and azathioprine. After two and a half years, peripheral eosinophilia had increased, and more of her skin had hardened. At that time, the therapy was modified to include corticoids, methotrexate, and penicillamine. It is of great importance to publicize these cases that allow us to gather experience and better treat our patients.

Controversy around the morphea

“…The active phase of the disease may or may not be characterized by peripheral eosinophilia, accelerated erythrocyte sedimentation rate (ESR), increased levels of C-reactive protein (CRP) and muscle enzymes, including creatine phosphokinase (CPK) and aldolase [13,14,16,25]. In addition, in the case of EF, the potential risk of coexistence of haematological disorders should be taken into account, which is estimated at 10-15% of all patients [19,26]. Therefore, it is recommended to perform morphology and electrophoresis with immunofixation [12][13][14].…”