RESUMO
BACKGROUND: The mucocutaneous clinical profile of patients with amyloidosis in Latin America has been scarcely reported. OBJECTIVE: To describe clinical characteristics of skin and mucosal manifestations in systemic amyloidosis in a tertiary care center in Mexico City. METHODS: A cross sectional, retrospective analytical study was performed in patients with systemic amyloidosis over a 15-year period. Statistical analysis was done. RESULTS: A total of 98 patients were included (53 [54%] men; overall median age = 49 years old). Acquired systemic immunoglobulin light chain amyloidosis (AL) was the most common (49%) type, followed by (24.5%) wild-type transthyretin amyloidosis (H-TTR) (24.5%), undetermined cases (21%), and reactive systemic amyloidosis (AA) (6.1%). There were mucocutaneous manifestations in 34.7% of cases, mostly multiple myeloma-related AL (ALMM). Head and neck was the most often affected site (38.2%), and purpuric macules were the most common morphology (44.1%). Mucocutaneous affectation was predominantly observed in AL (50.0%) compared to other types (20.0%) of amyloidosis (P = 0.01). Likewise, involvement of organs was also significantly different among the diverse amyloidosis types (P < 0.05). The most frequent comorbidities were hypertension (18.3%) and hypothyroidism (18.3%). CONCLUSION: The clinical spectrum of manifestations in amyloidosis is wide. Involvement of skin and mucosa in amyloidosis is common; significant differences were observed concerning distribution of mucocutaneous amyloid manifestations among the different types of amyloidosis.
Assuntos
Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Mucosa/patologia , Mieloma Múltiplo/patologia , Pele/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Estudos Transversais , Feminino , Humanos , Hipertensão/epidemiologia , Hipotireoidismo/epidemiologia , Amiloidose de Cadeia Leve de Imunoglobulina/epidemiologia , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Mieloma Múltiplo/epidemiologia , Estudos Retrospectivos , Centros de Atenção Terciária/estatística & dados numéricosRESUMO
Systemic light chain amiloydosis is a rare disease. Due to its typical cutaneous lesions, dermatologists play an essential role in its diagnosis. Clinical manifestations vary according to the affected organ and are often unspecific. Definitive diagnosis is achieved through biopsy. We report a patient with palpebral amyloidosis, typical bilateral ecchymoses and cardiac involvement, without plasma cell dyscrasia or lymphomas. The patient died shortly after the diagnosis.
Assuntos
Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Dermatopatias/diagnóstico , Adulto , Biópsia , Evolução Fatal , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Dermatopatias/patologiaRESUMO
Abstract: Systemic light chain amiloydosis is a rare disease. Due to its typical cutaneous lesions, dermatologists play an essential role in its diagnosis. Clinical manifestations vary according to the affected organ and are often unspecific. Definitive diagnosis is achieved through biopsy. We report a patient with palpebral amyloidosis, typical bilateral ecchymoses and cardiac involvement, without plasma cell dyscrasia or lymphomas. The patient died shortly after the diagnosis.