Morphoea-like plaques revealing an eosinophilic (Shulman) fasciitis

Abstract

Eosinophilic fasciitis (EF) is a connective-tissue disease characterized by thickened fascia. Involvement of the dermis may occur during the progression of the disease, characterized clinically by morphoea-like plaques (MLPs). This more superficial feature of EF carries a poor prognosis, suggestive of refractory EF that requires intensive therapy. We report a case in which morphoea-like plaques occurred before the EF. This case and previous cases of MLPs show some differences between MLPs and classic morphoea. Clinically, MLPs present with ill-defined brown plaques, with no lilac ring or ivory colour. Histologically, there is a more marked inflammatory infiltrate and an increase in the number of eosinophils. The discovery of MLPs at an early stage should prompt examination of the fascia with magnetic resonance imaging or surgical biopsy, and aggressive treatment.