Shulman syndrome: An atypical presentation of a rare disease
Main Article Content
Keywords
Steroids, Scleroderma, Systemic, Fasciitis, Shulman’s Syndrome
Abstract
Background: Eosinophilic fasciitis is a rare scleroderma-like disorder. Most of the available information comes from case reports or small case series. It is characterized by fascial thickening, acute pain, skin induration, joint contractures, and, anecdotally, visceral involvement. Diagnosis is confirmed through deep biopsy, although magnetic resonance imaging may be useful in inconclusive cases.
Clinical case: We describe the case of a previously healthy 32-year-old man male with a history of recurrent minor trauma to the lumbar and gluteal regions. He developed acute-onset pain and progressive deep induration in the affected regions, which later extended symmetrically to all four extremities. Initial magnetic resonance imaging suggested lymphedema; however, given the high clinical suspicion, images were re-evaluated, revealing findings consistent with fasciitis. Treatment with corticosteroids and methotrexate was initiated, resulting in rapid improvement of residual pain and gradual reduction of deep induration.
Conclusion: This case highlights the heterogeneous presentation of eosinophilic fasciitis and underscores the importance of including it in the differential diagnosis of scleroderma-like disorders, as well as the role of magnetic resonance imaging in ambiguous cases.
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