Type I acquired cutis laxa: Report of a unique progressive case and short review.
Am J Med Sci
; 367(4): 268-273, 2024 04.
Article
em En
| MEDLINE
| ID: mdl-38278360
ABSTRACT
Cutis laxa is a rare connective tissue disorder, characterized by a reduced number and abnormal properties of elastic fibers throughout the dermis, creating a clinical appearance of premature aging. It can be subdivided into inherited and acquired, the latter rarer than the former, and skin involvement may be localized or generalized. The etiology of acquired cutis laxa (ACL) remains unknown and there is no definitive treatment. We present the case of a 30-year-old man diagnosed with type I ACL with progressive systemic involvement at the renal, pulmonary, and digestive levels. Histological analysis of the skin revealed reduction and fragmentation of elastic fibers. Immunosuppressive treatment was started with prednisone, cyclophosphamide, and rituximab, with which a complete response to proteinuria was achieved and the progression of lung damage was limited. Autoimmune, infectious, and neoplastic diseases were ruled out.
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Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Cútis Laxa
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Am J Med Sci
Ano de publicação:
2024
Tipo de documento:
Article