Eosinophilic fasciitis: an atypical presentation of a rare disease

Rev Assoc Med Bras (1992). 2019 Mar;65(3):326-329. doi: 10.1590/1806-9282.65.3.326. Epub 2019 Apr 11.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Edema / pathology
  • Eosinophilia / diagnostic imaging
  • Eosinophilia / drug therapy
  • Eosinophilia / pathology*
  • Fasciitis / diagnostic imaging
  • Fasciitis / drug therapy
  • Fasciitis / pathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Rare Diseases
  • Skin / pathology
  • Treatment Outcome

Supplementary concepts

  • Eosinophilic Fasciitis