Systemic amyloidosis presenting as mucocutaneous bullous lesions
aGovernment Medical College, Srinagar and bSher-i-Kashmir Institute of Medical Sciences (Medical College Hospital), Srinigar, Jammu and Kashmir, India
Ahmad QM, Sultan SJ, Shah IH, Sameema F. Systemic amyloidosis presenting as mucocutaneous bullous lesions. Hematol Oncol Stem Cell Ther 2009; 2(3): 418-421
Abstract
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 l 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.
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