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Abstract

Bullous amyloidosis is a rare presentation of amyloidosis that is not often high on the differential diagnosis for a bullous dermatosis without additional key findings. A male in his 60s with a history of hypertension, type II diabetes mellitus, and sickle cell trait presented to the emergency department with a 7-month history of progressive painful lesions on his face, neck, and axilla. The patient had a history of working in the textile industry with exposure to industrial grade chemicals. Physical examination revealed soft, fleshy, red infiltrative nodules involving the cheeks, lips, and oral mucosa, along with fragile, thin bullae scattered on the anterior neck and axilla. Punch biopsies were obtained from the oral mucosa, cheek, and axilla, revealing a mixed inflammatory infiltrate, pseudoepitheliomatous hyperplasia, and pauci-inflammatory subepidermal bullae, respectively. In addition, pink amorphous material was noted in all specimens with positive Congo red staining and partial birefringence on polarized light microscopy. Mass spectrometry confirmed AL (amyloid light-chain) amyloidosis with lambda light-chain predominance. Serum immunofixation identified an IgG lambda monoclonal paraprotein. Cardiac biomarkers were elevated, suggesting systemic involvement. Further bloodwork demonstrated a large paraprotein gap, raising concern for concomitant multiple myeloma. The patient was started on an appropriate chemotherapy regimen for multiple myeloma with systemic AL amyloidosis. While this patient had plaques and nodules classic for a depositional process, the bullae present on the neck and axillae highlight a more unusual clinical variant of amyloidosis that can also assist in uncovering the underlying diagnosis in the appropriate clinical context.