Primary systemic amyloidosis

Abstract

A 45-year old male had nephrotic syndrome of 2 years duration and multiple, asymptomatic, small, yellowish papules on eyelids, nasolabial folds and perioral region since 6 months. He also had progressive weakness, fatigue, breathlessness and joint pains. His voice was hoarse. Macroglossia and beaded vocal cords were present. There was a history of exsanguinating bleeding following a kidney biopsy done 6 months earlier.Proteinuria was present but Bence-Jones proteins were absent. Skin biopsy of lesional skin showed an eosinophilic, amorphous, fissured mass distending the dermal papillae and around blood vessels and appendages. Congo red stain was negative for amyloid, but crystal violet stain demonstrated amyloid in lesional as well as normal appearing skin. Electron microscopy confirmed the same. The patient deteriorated quickly and succumbed to gross haematemesis.