Hemorrhagic bullous amyloidosis
Madigan L, Lim H. Hemorrhagic bullous amyloidosis. Journal of the American Academy of Dermatology 2017; 76(6 Supplement 1):AB152.
Journal of the American Academy of Dermatology
We present the case of a 66-year-old African American woman with a history of chronic kidney disease and a rare subtype of multiple myeloma who was admitted in the setting of a gastrointestinal bleed and new onset diastolic heart failure. Dermatology was consulted for ''evaluation of intertrigo.'' During the initial encounter, she endorsed a 6-month history of progressive erythema and new onset blistering within the skin folds. On examination, macular purpura and several large, hemorrhagic bullae were appreciated in intertriginous areas. A punch biopsy was performed which revealed amorphous, pink deposits within the dermal papilla and surrounding blood vessels; a Congo red stain was positive. Laboratory studies were notable for anemia and an elevated lambda light chain level (2040 mg/L). Further cardiac imaging revealed thickening of the left ventricular wall and functional anomalies consistent with cardiac amyloidosis. The patient was diagnosed with systemic amyloidosis in the setting of recurrent multiple myeloma. Upon reviewing the literature, varied treatment regimens have been reported for this uncommon manifestation. In 2012, a group of investigators reported success with an aggressive 5-drug combination chemotherapy regimen and subsequent stem cell transplantation. The patient presented elected to forgo treatment and was transitioned to hospice care.
6 Supplement 1