Scleroderma – An Unusual Presenting Feature of Primary Systemic Amyloidosis

The case presented in this report is one of 45-year-old female with dysphagia and swelling in the neck since 2 months, breathlessness, history of inability to open the mouth and course of events resembled scleroderma and diagnosis of primary systemic amyloidosis could not be established until autopsy. At autopsy skin, oesophagus, salivary glands, tongue and all the parenchymal organs like spleen, kidney, liver, pancreas, lungs and thyroid showed amyloid deposition which exhibited apple green birefringence by congo red stain and was resistant to potassium permanganate test (KMnO4). It was thus labelled as primary systemic amyloidosis. The purpose of this case report is to reiterate the importance of a high index of suspicion in the unusual clinical presentations of primary generalized amyloidosis.