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- Title
Epidermólisis ampollosa en niños: un estudio retrospectivo en un hospital de referencia.
- Authors
Isabel Araiza-Atanacio, María; Gris-Calvo, Judith; Juana Piña-Ramírez, María; Francisco Cadena-León, José; Teja Ángeles, Eduardo de la; Varón-Munar, Diana; Teresa García-Romero, María
- Abstract
Background: Epidermolysis bullosa (EB) is a genodermatosis caused by mutations in the proteins of the dermal-epidermal junction, altering the epithelial cohesion, and generating blisters and shedding of skin and mucous membranes. Objective: To describe the demographic and clinical characteristics, as well as the main complications of patients with EB attended at the National Institute of Pediatrics, in Mexico City. Method: An observational, descriptive, retrospective and cross-sectional study was conducted in patients under 18 years of age with diagnosis of EB. Patients with incomplete, purged or archived records were excluded. Results: We included 35 patients, 17 men and 18 women with an average age of 8.94 ± 4.9 years. Patients were classified as dystrophic EB (71.4%), EB simplex (17%), junctional EB (2.9%) and Kindler syndrome (2.9%). All patients presented skin manifestations, followed by manifestations in oral mucosa (74.3%), nutritional (54.2%), gastrointestinal (51.4%), hematological (40%), ophthalmological (37.1%), musculoskeletal (34.2%) and psychosocial symptoms (34.2%). The degree of severity was variable according to the subtype; junctional EB and dystrophic EB are those that generate greater affection and comorbidity. Conclusions: EB is a serious multisystem genetic disease, which is why it requires an early diagnosis and a timely detection of complications.
- Publication
Revista Medica del IMSS, 2020, Vol 58, Issue 5, p583
- ISSN
0443-5117
- Publication type
Article
- DOI
10.24875/RMIMSS.M20000088