Dilemmas in the Management of Digital Ulcers in Systemic Sclerosis-Mixed Connective Tissue Disease: Lessons From a Case Report
Abstract
Systemic sclerosis (SSc) is a rare autoimmune disorder marked by fibrosis and microvascular injury, commonly manifesting as digital ulcers and skin thickening. Lower limb ulcers are uncommon but can contribute to significant morbidity, need prolonged healing, and carry the risk of progression to necrosis or amputation, particularly in systemic sclerosis-mixed connective tissue disease (SSc-MCTD). We report a case of a 57-year-old woman with Raynaud's phenomenon, inflammatory small-joint arthritis, alopecia, photosensitive rash, and exertional dyspnea. Despite improvement in arthritis with disease-modifying antirheumatic therapy, a lower limb ulcer progressed while she was on vasoactive therapy (α-adrenergic blockade, phosphodiesterase-5 inhibition, endothelin-receptor antagonism) and immunomodulation, culminating in necrosis and pain that required amputation of the great toe and subsequently the forefoot. Laboratory evaluation revealed systemic inflammation and dyslipidemia, while vascular imaging demonstrated both microvascular and macrovascular compromise. Histopathology confirmed acute inflammatory changes without primary vasculitis. Rheumatology evaluation and serology established a diagnosis of SSc-MCTD, after which management was refocused on targeted vasodilation, immunomodulatory therapy, and multidisciplinary wound care. This case underscores the central role of vasculopathy in SSc-MCTD, highlights the limited efficacy of surgical intervention without optimized…
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