Perianal purpuric plaques revealing an amyloid light-chain amyloidosis: case report and review of the literature.
Dermatol Online J
; 25(1)2019 Jan 15.
Article
em En
| MEDLINE
| ID: mdl-30710905
ABSTRACT
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Púrpura
/
Dermatopatias Metabólicas
/
Amiloidose de Cadeia Leve de Imunoglobulina
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Limite:
Aged
/
Humans
/
Male
Idioma:
En
Revista:
Dermatol Online J
Assunto da revista:
DERMATOLOGIA
Ano de publicação:
2019
Tipo de documento:
Article