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Aplasia congênita da cútis em membros inferiores: relato de caso
| Content Provider | Semantic Scholar |
|---|---|
| Author | Almeida, Kleder Gomes De Campos, Patrícia Costa Costa, Gabriel Rahal Nokarya, Paulete Yuri Oliveira, Gustavo De Sousa Marques |
| Copyright Year | 2015 |
| Abstract | INTRODUCTION Aplasia cutis congenita (ACC) is a rare disease characterized by the absence of a part of the skin at birth that may be either localized or widespread. The incidence of this disease is 0.1 per 100,000 births. It mainly involves the scalp alone in 60% of the cases. The causes are unclear; however, genetic factors, teratogens (alcohol, cocaine, marijuana, heroin, misoprostol, methimazole, carbimazole, congenital herpes simplex, congenital varicella, and others), impaired blood supply to the skin, trauma, and amniotic band and chromosomal disorders (trisomy 13) are associated with the wounds. CASE REPORT: A female patient was delivered vaginally at 36 weeks 3 days of gestation, with appropriate weight for the gestational age. She was referred to our service after 7 days of birth, presenting total absence of skin integument on both legs, with impairment of approximately 17% of the body surface according to the Lund and Browder chart. The malformation consisted of the total absence of skin and subcutaneous tissue. Given the extent and localization of the wound, treatment with a partial skin graft was elected, thereby filling the totality of the defect. CONCLUSION: Owing to the rarity of ACC and the small number of patients in the series published in the literature, standardization of treatment is still incipient. Currently, only recommendations are available. Further studies are needed in order to investigate the etiology of the disease and to evaluate treatment methods. INTRODUCAO A Aplasia Congenita da Cutis (ACC) e uma doenca rara caracterizada pela ausencia de uma parte da pele ao nascimento, seja em area localizada ou generalizada. A incidencia e de 0,1 a cada 100.000 nascimentos, tendo como acometimento principal o couro cabeludo, ocorrendo de forma isolada nesta localizacao em 60% dos casos. As causas nao sao claras, mas fatores geneticos, teratogenos (alcool, cocaina, maconha, heroina, misoprostol, metimazol, carbimazol, herpes simples congenito, varicela congenita, etc.), comprometimento da irrigacao sanguinea para a pele, trauma, bandas amnioticas e desordens cromossomicas (trissomia do 13) estao associados com as lesoes. RELATO DO CASO: Paciente do sexo feminino, nascida de parto vaginal com 36 semanas e tres dias, e peso adequado para a idade gestacional. Referenciada ao nosso servico apos sete dias de nascimento, constando ausencia total do tegumento cutâneo em ambas as pernas, com acometimento de aproximadamente 17% da superficie corporea, segundo a tabela de queimados de Lund e Browder. A ma formacao consistia na ausencia total de pele e tecido celular subcutâneo. Face a extensao e localizacao da lesao, optou-se pelo tratamento por enxerto de pele parcial, permitindo, dessa forma, preencher a totalidade do defeito. CONCLUSAO: Devido a raridade da ACC e ao pequeno numero de pacientes nas series publicadas na literatura, a padronizacao do tratamento ainda e incipiente e o que existe sao recomendacoes. Ainda sao necessarios estudos que abordem desde a etiologia da doenca ate a avaliacao dos metodos de tratamento. |
| Starting Page | 143 |
| Ending Page | 147 |
| Page Count | 5 |
| File Format | PDF HTM / HTML |
| Volume Number | 30 |
| Alternate Webpage(s) | http://www.rbcp.org.br/export-pdf/1614/v30n1a24.pdf |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |