Systemic Sclerosis Presenting With Long-Standing Peripheral Neuropathy and Delayed Multisystem Manifestations: A Case Report.

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disorder characterized by immune dysregulation, microvascular injury, and progressive fibrosis involving the skin and internal organs. Although Raynaud phenomenon and cutaneous sclerosis are the hallmark manifestations, atypical presentations may significantly delay diagnosis. Peripheral neuropathy is an uncommon neurological manifestation and rarely precedes the diagnosis of SSc by several years. We report the case of a 62-year-old female who presented with a 20-year history of progressive distal paresthesia involving both upper and lower limbs, followed by recurrent lower limb ulceration and progressive unilateral lower-limb swelling associated with skin thickening and deformity. Nearly two decades after the onset of neurological symptoms, she developed progressive dysphagia, constitutional symptoms, exertional dyspnea, and pigmentary skin changes. Evaluation demonstrated positive antinuclear antibodies with strong anti-Ro52 and anti-RNP/Sm antibodies, mixed sensorimotor peripheral neuropathy with bilateral carpal tunnel syndrome, pulmonary arterial hypertension, interstitial lung disease, moderate pericardial effusion, gastric antral vascular ectasia, and histopathological evidence of dermal sclerosis. She was diagnosed with SSc with overlap autoimmune features and managed through a multidisciplinary approach involving rheumatology, pulmonology, gastroenterology, rehabilitation medicine, and physiotherapy. This case illustrates an unusual neuropathy-predominant presentation in which peripheral nerve involvement preceded classical systemic manifestations by almost two decades. It highlights the importance of considering connective tissue diseases in patients presenting with chronic unexplained peripheral neuropathy, recurrent trophic ulceration, or progressive localized skin sclerosis. Early recognition of such atypical presentations may facilitate timely diagnosis, comprehensive organ screening, and administration of disease-modifying therapy before irreversible organ damage develops.