Epidermolysis bullosa in Peru: Clinical and epidemiological study of patients treated in a national reference pediatric hospital, 1993-2015

Rosario Torres-Iberico, Patricia Palomo-Luck, Gilmer Torres-Ramos, Roxana Lipa-Chancolla

Research output: Contribution to journalArticle

Abstract

© 2017, Instituto Nacional de Salud. All rights reserved. Objectives. To describe the clinical and epidemiological characteristics of patients diagnosed with epidermolysis bullosa (EB) at the Instituto Nacional de Salud (INSN) in Lima, Peru; a National Reference Center for this disease. Materials and methods. Observational, descriptive and transversal study. We reviewed the clinical histories and laboratory tests of patients diagnosed with EB treated in INSN from 1993 to 2015. Results. 93 patients were registered. The average age was 7.9 ± 5.6 years; 53.8% (n = 50) were boys. Clinical forms corresponded to dystrophic EB with 41 (44.1%) cases, simple EB with 39 (41.9%), union EB cases with 8 (8.6%) and Kindler syndrome with 4 (4.3%) cases. The clinical form could not be identified in a case. A total of 48 cases (51.6%) came from Lima and Callao, and 45 cases (48.4%) from other provinces of the country. Extracutaneous manifestations involved gastrointestinal (44.1%), ocular (37.6%), odontogenic (87.1%), and nutritional (79.6%) involvement, as well as pseudosindactilia (16.1%). Chronic malnutrition (71.6%), acute malnutrition (17.6%) and anemia (62.4%) were found. Mortality corresponded to 6 cases (6.5%). Conclusions. 93 cases of EB were reported in INSN, the predominant clinical presentation was the dystrophic form.
Original languageAmerican English
Pages (from-to)201-208
Number of pages8
JournalRevista Peruana de Medicina Experimental y Salud Publica
DOIs
StatePublished - 1 Apr 2017
Externally publishedYes

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