Systemic amyloidosis presenting as mucocutaneous bullous lesions

A 65-year-old male presented with hemorrhagic bullous skin lesions with purpura and ecchymoses. There was increased skin fragility with a strongly positive Nikolsky sign. Histopathology of the skin revealed large amounts of amyloid deposits in the dermis with a positive Congo Red staining around the dermal vessels. Examination and tests in this patient also revealed anemia, hepatomegaly, infiltrative cardiomyopathy, polyneuropathy and immunoglobulin lamda deposition, favoring a diagnosis of primary amyloidosis [AL type]. The present case is reported in view of the rarity of the bullous variant of primary systemic amyloidosis as well as presence of mucosal lesions and a positive Nikolsky sign