An Unusual Presentation of a Rare Disease: Eosinophilic Fasciitis

一种罕见疾病的不寻常表现:嗜酸性筋膜炎

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Abstract

Eosinophilic fasciitis is an uncommon disorder presenting with diffuse fasciitis and peripheral eosinophilia. Due to the rarity of this disorder and limited literature, its diagnosis and treatment are often delayed. We present the case of a young male wherein the diagnosis of eosinophilic fasciitis was initially delayed due to an atypical presentation. However, after the diagnosis was confirmed, the patient was successfully managed with oral corticosteroids. A well-written informed consent was obtained from the patient prior to the preparation of this manuscript. An 18-year-old right-hand dominant male presented with a sudden onset, progressive, non-traumatic, left forearm swelling associated with a weak hand grip. The swelling was tender on examination with a local rise in temperature. Radiographs taken at the time of presentation revealed no osseous pathology. As the initial blood investigations were suggestive of a localized inflammatory pathology involving the forearm, the patient was managed with non-steroidal anti-inflammatory drugs and analgesics. He returned 6 months later with a recurrence of the symptoms. A magnetic resonance imaging scan of the left forearm was performed to further investigate the pathology and it was suggestive of a diffuse plaque-like thickening involving the myofascial layer of the muscles. Blood investigations showed peripheral eosinophilia, raised immunoglobulin G count, and raised inflammatory markers. A full-thickness forearm biopsy showed the presence of lymphocytic infiltration. A diagnosis of eosinophilic fasciitis was suspected and the patient was managed with oral corticosteroids, given as a tapering dose. Following this, the patient had symptomatic improvement with the resolution of the deranged blood parameters. He was asymptomatic at the latest follow-up of 4 years.

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