CPC02 Eosinophilic fasciitis evolving into severe generalized morphoea: a rare spectrum of fibrosing immune disease

Abstract

Eosinophilic fasciitis and generalized morphoea are uncommon fibrosing inflammatory disorders that share overlapping clinical and pathological features, yet they are traditionally regarded as distinct entities. We report a case that illustrates progression across this spectrum and highlights the diagnostic and therapeutic challenges that arise when disease evolves over time. A 69-year-old woman presented with skin induration, musculoskeletal pain and functional decline following a recent viral infection. Her background history included thyroidectomy and treated uterine carcinoma 10 years previously. There was no history of chemical exposure, trauma or unaccustomed exercise. Prior to presentation she had developed marked hypereosinophilia (23%), which resolved after extensive investigation, and she also had a raised IgG4 of uncertain significance. Autoimmune screening, including connective tissue disease, scleroderma and myositis panels, was negative. Magnetic resonance imaging was suggestive of eosinophilic fasciitis; however, full-thickness skin and fascial biopsy was nondiagnostic. A working diagnosis of eosinophilic fasciitis was made, and high-dose oral prednisolone was commenced, followed by six cycles of cyclophosphamide, but skin thickening and functional impairment progressed. Examination demonstrated peau d’orange changes and a positive groove sign of the medial thighs, with no digital ulceration, calcinosis or periungual erythema. Over subsequent months she developed widespread erythematous–violaceous discoloration involving the upper limbs, chest and ankles, evolving into extensive symmetrical plaque-type sclerosis sparing the hands, forehead and cheeks. In light of this progression, histological slides were reviewed at a dermatopathology meeting, and the consensus diagnosis was in keeping with morphoea. Treatment was escalated to methotrexate and abatacept, although methotrexate was subsequently changed to mycophenolate mofetil. This case demonstrates dynamic evolution from eosinophilic fasciitis to severe generalized morphoea, supporting the concept of a unified fibrosing immune-mediated disease spectrum. It highlights the limitations of biopsy, the importance of ongoing clinicopathological reassessment, and the need to recognize progression despite aggressive immunosuppression.