A carregar...
Publicação
Colagenose perfurante reativa adquirida : a propósito de um caso clínico
| Nome: | Descrição: | Tamanho: | Formato: | |
|---|---|---|---|---|
| 475.46 KB | Adobe PDF |
Orientador(es)
Resumo(s)
A Colagenose Perfurante Reativa Adquirida (CPRA) é uma doença dermatológica rara, que integra um grupo maior de manifestações cutâneas, as dermatoses perfurantes, que se caracterizam pela eliminação transepidérmica de diferentes constituintes dérmicos. Sendo que, no caso da patologia em causa, é a eliminação de colagénio que a distingue das restantes entidades. A CPRA é essencialmente descrita em associação com uma grande variedade de patologias sistémicas, sejam elas entidades benignas ou neoplásicas, o que dificulta não só o entendimento da sua fisiopatologia, grande parte da qual ainda por esclarecer, como também a sua identificação clínica e diagnóstico. Clinicamente, manifesta-se pela presença de prurido intenso e generalizado e aparecimento de pápulas ou nódulos umbilicados e hiperqueratósicos, com localização preferencial no tronco e extremidades. Por forma a rever e estruturar a literatura existente referente ao tema, e por descrever uma nova associação com doença sistémica, apresenta-se o caso de uma doente, sexo feminino, 85 anos, internada por um quadro de alterações analíticas do perfil hepático e apresentando como sintomatologia uma dermatose pruriginosa e perda ponderal, a quem foi diagnosticada uma CPRA, no contexto de estenose biliar iatrogénica póscolecistectomia, excluindo-se a possibilidade de síndrome paraneoplásico.
Acquired reactive perforating collagenosis (ARPC) is a rare dermatological disease that belongs to a broader group of skin manifestations known as perforating dermatoses. These conditions are characterized by the transepidermal elimination of different dermal constituents. In ARPC, the elimination of collagen distinguishes it from other entities. ARPC is primarily described in association with a wide variety of systemic diseases, whether benign or neoplastic. This not only complicates the understanding of its pathophysiology, much of which is still unclear, but also its clinical identification and diagnosis. Clinically, it presents as intense and generalized pruritus, accompanied by the appearance of umbilicated and hyperkeratotic papules or nodules, predominantly located on the trunk and extremities. We present the case of an 85-year-old female patient, who was admitted to the hospital due to alterations in hepatic profile laboratory results and presented with pruritic dermatosis and weight loss, so as to review and summarize the existing literature on the subject and to give testimony of a new association with a systemic disease. The patient was diagnosed with ARPC in the context of iatrogenic biliary stricture following cholecystectomy, with paraneoplastic syndrome being ruled out.
Acquired reactive perforating collagenosis (ARPC) is a rare dermatological disease that belongs to a broader group of skin manifestations known as perforating dermatoses. These conditions are characterized by the transepidermal elimination of different dermal constituents. In ARPC, the elimination of collagen distinguishes it from other entities. ARPC is primarily described in association with a wide variety of systemic diseases, whether benign or neoplastic. This not only complicates the understanding of its pathophysiology, much of which is still unclear, but also its clinical identification and diagnosis. Clinically, it presents as intense and generalized pruritus, accompanied by the appearance of umbilicated and hyperkeratotic papules or nodules, predominantly located on the trunk and extremities. We present the case of an 85-year-old female patient, who was admitted to the hospital due to alterations in hepatic profile laboratory results and presented with pruritic dermatosis and weight loss, so as to review and summarize the existing literature on the subject and to give testimony of a new association with a systemic disease. The patient was diagnosed with ARPC in the context of iatrogenic biliary stricture following cholecystectomy, with paraneoplastic syndrome being ruled out.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2023
Palavras-chave
Colagenose perfurante reativa adquirida Dermatoses perfurantes Estenose biliar Síndrome paraneoplásico Prurido