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DOI: 10.1055/s-0041-1724066
Pediatric Metastatic Crohn's Disease
Doença de Crohn metastática pediátrica
Abstract
Crohn's disease (CD) is a chronic, relapsing, idiopathic condition, characterized by granulomatous, transmural inflammation of the gastrointestinal tract, which can affect its entire length, from mouth to anus. Metastatic Crohn's disease (MCD) is a rare form of skin involvement and is defined by skin lesions without contiguity with the gastrointestinal tract. A 9-year-old patient presented with gastrointestinal complaints and gross skin lesions in the vulva and perianal region. The diagnosis of Crohn's disease was made when the patient was 11 years old, after being evaluated by the colorectal surgeon. Treatment was started with a “top-down” approach, with a sustained response for four years. Afterwards, there was a relapse of the skin disease in previously normal areas, without overt symptoms. Treatment consisted of steroids and local infiltration of infliximab, without improvement. A year later, there was a rapid progression of the skin lesions, and the drug changed to adalimumab, also without response and worsening of the skin lesions. The patient was admitted to the hospital and intravenous steroids were initiated, along with surgical debridement of the lesions. After some improvement, ustekinumab was initiated with satisfactory response. Pediatric MCD has an important impact on the patient's quality of life, with influences on growth and social development.
Resumo
A doença de Crohn é uma condição idiopática crônica, recidivante, caracterizada por inflamação granulomatosa transmural do trato gastrointestinal, que pode afetar toda a sua extensão, da boca ao ânus. A doença de Crohn metastática (DCM) é uma forma rara que envolve a pele, e é definida por lesões cutâneas sem contiguidade com o trato gastrointestinal. Uma paciente de 9 anos de idade apresentou queixas gastrointestinais e lesões cutâneas grosseiras na vulva e na região perianal. O diagnóstico de doença de Crohn foi feito quando a paciente tinha 11 anos, após avaliação do coloproctologista. O tratamento foi então iniciado com uma abordagem “top-down”, com uma resposta mantida por quatro anos. Posteriormente, houve recidiva da doença de pele em áreas anteriormente normais, sem sintomas evidentes. O tratamento consistiu em corticoide e infiltração local de infliximabe, sem melhora. Um ano depois, houve rápida progressão das lesões cutâneas, sendo a medicação alterada para adalimumabe, também sem resposta e com piora das lesões cutâneas. A paciente foi internada e iniciado tratamento com corticoide intravenoso, juntamente com o desbridamento cirúrgico das lesões. Após alguma melhora, o ustecinumabe foi iniciado com resposta satisfatória. A DCM pediátrica tem um impacto importante na qualidade de vida do paciente, com influências no crescimento e no desenvolvimento social.
Note
The objective of the present paper is to address a case of metastatic CD, a rare manifestation of Crohn's in a pediatric patient.
We reviewed the medical records, from the patient's first consultation, with our group. We also did a literature review, searching for publications regarding MCD and CD in the pediatric population.
Publikationsverlauf
Eingereicht: 30. April 2020
Angenommen: 07. Juni 2020
Artikel online veröffentlicht:
19. März 2021
© 2021. Sociedade Brasileira de Coloproctologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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