Skin Fragility, Diffuse Ecchymosis, and Blisters on a Yellowish Waxy Base
May 2016
in “
Journal of The American Academy of Dermatology
”
TLDR A woman's skin symptoms led to a diagnosis of systemic AL amyloidosis, but she died from sepsis shortly after.
In 2016, a case report highlighted the presentation of systemic AL amyloidosis, a condition characterized by immunoglobulin light chain deposits in vital organs, which can lead to organ dysfunction and death. Cutaneous involvement occurs in about 40% of patients, but bullous lesions are rare. The report detailed a 74-year-old woman with skin fragility and non-itching blisters on yellowish waxy plaques, which were initial presentations of systemic AL amyloidosis. Despite normal serum and urine protein electrophoresis, immunofixation electrophoresis of urine detected monoclonal kappa chains, and a skin biopsy with Congo red stain confirmed the diagnosis. Unfortunately, the patient died from sepsis two weeks later. This case emphasizes the importance of considering bullous amyloidosis in the differential diagnosis of bullous dermatoses to ensure prompt diagnosis and treatment.
