A Rare Cutaneous Manifestation of Systemic Sclerosis

A 43-year-old male with a history of intravenous drug use and alcohol consumption presented to the emergency department with three-month history of failure to thrive. The patient exhibited a constellation of constitutional symptoms including cough, weight loss, fatigue, decreased appetite, nausea and vomiting. The skin examination revealed multiple subcutaneous hyperpigmented, indurated plaques and nodules on the trunk and arms. Laboratory evaluation revealed abnormal autoimmune tests, anaemia, elevated inflammatory markers and radiological evidence of interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). An excisional biopsy from a skin lesion demonstrated dermal sclerosis consistent with scleroderma. The patient was diagnosed with diffuse systemic scleroderma with cutaneous findings consistent with nodular or keloidal scleroderma variant. This case highlights a rare cutaneous variant of systemic scleroderma called nodular or keloidal scleroderma.

Nodular scleroderma can be a diagnostic challenge due to its rarity and wide clinical presentation, which can mimic other medical conditions such as keloid or hypertrophic scar, storiform collagenoma, sclerotic dermatofibroma or sclerosing perineuroma.Clinicians should have a high degree of suspicion for nodular scleroderma when a patient presents with firm nodular or keloidal skin lesions, to diagnose and treat it appropriately.It is important to recognise nodular scleroderma early because timely and accurate diagnosis is crucial for appropriate management to prevent severe debilitation and scleroderma-related complications, and to improve patient outcomes.