Nephrogenic Systemic Fibrosis: A Rare Contemporary Case After a Decade of Declining Incidence

ABSTRACT Nephrogenic systemic fibrosis (NSF) is a rare fibrosing disorder associated with exposure to gadolinium‐based contrast agents in patients with renal impairment. Although its incidence has markedly declined since the restriction of high‐risk linear gadolinium agents, sporadic cases may still occur. We report the case of an 82‐year‐old woman with hypertension and stage 3a chronic kidney disease secondary to nephroangiosclerosis who developed rapidly progressive cutaneous induration after undergoing magnetic resonance imaging with intravenous gadolinium contrast. She presented with symmetric hyperpigmented and hypopigmented plaques over the neck, trunk, buttocks, abdomen, flexural regions, and proximal limbs, sparing the face. The affected skin was firm, indurated, and difficult to pinch. Histopathological examination of two biopsies revealed marked dermal and hypodermal collagen sclerosis, loss of adnexal structures, and preservation of elastic fibres. A multidisciplinary assessment excluded systemic involvement. The temporal relationship with gadolinium exposure, compatible histopathological findings, and clinical presentation supported the diagnosis of NSF. The patient declined systemic therapy and was managed with topical corticosteroids and gentle heliotherapy, achieving partial improvement after 4 months. NSF remains a clinically significant entity due to its potential morbidity, diagnostic overlap with scleroderma, and the need for strict preventive measures in patients with renal dysfunction. This case underscores the relevance of maintaining awareness of NSF, even today, more than a decade after its incidence markedly decreased.