Perianal purpuric plaques revealing an amyloid light-chain amyloidosis: case report and review of the literature

• Type: Article in International Scientific Journal
• Date: 2019-01-01

Authors

Lopes, JM (Author) FMUP

Journal

Title: Dermatology Online Journal

Vol. 25 No. 6

Pages: 1-4

ISSN: 1087-2108

Publisher: University of California Press

Indexing

Scopus - 1 Citation

Other information

Authenticus ID: P-00Q-6GG

Abstract (EN): Systemic immunoglobulin light chain amyloidosis is the most common and severe type of amyloidosis. There is an abnormal fibrillary protein deposition in tissues that leads to progressive and irreversible organ dysfunction. The most commonly affected organs are kidney and heart. Although rare, cutaneous manifestations may be the first clinical sign of the disease and usually present as hemorrhagic lesions, such as purpura, petechiae, and ecchymosis. We present a 71-year-old man that presented to our department because of exuberant purpuric plaques in the anogenital area as the first manifestation of an amyloid light-chain (AL) amyloidosis. The multi-organ involvement in addition to rapid clinical deterioration precipitated the patient's death four months later.

Language: English

Type (Professor's evaluation): Scientific