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

Authors

  • Rosario Torres-Iberico Instituto Nacional de Salud del Niño. Lima, Perú. Médico dermatólogo
  • Patricia Palomo-Luck Instituto Nacional de Salud del Niño. Lima, Perú. Licenciada en Nutrición, especialista en Nutrición Clínica
  • Gilmer Torres-Ramos Instituto Nacional de Salud del Niño. Lima, Perú. Odontólogo, especialista en Odontopediatría
  • Roxana Lipa-Chancolla Instituto Nacional de Salud del Niño. Lima, Perú. Médico patólogo

DOI:

https://doi.org/10.17843/rpmesp.2017.342.2484

Keywords:

Epidermolysis bullosa, Epidermolysis bullosa dystrophica, junctional, Epidermolysis bullosa simplex

Abstract

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.

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Author Biographies

  • Rosario Torres-Iberico, Instituto Nacional de Salud del Niño. Lima, Perú. Médico dermatólogo
    Médico asistente del Servicio de Dermatologia del Instituto Nacional de Salud del NIño Breña
  • Patricia Palomo-Luck, Instituto Nacional de Salud del Niño. Lima, Perú. Licenciada en Nutrición, especialista en Nutrición Clínica
    Lic. en Nutrición - Nutricionista INSN
  • Gilmer Torres-Ramos, Instituto Nacional de Salud del Niño. Lima, Perú. Odontólogo, especialista en Odontopediatría
    Odontopediatra del Servicio de Odontología del INSN
  • Roxana Lipa-Chancolla, Instituto Nacional de Salud del Niño. Lima, Perú. Médico patólogo
    Médico Patologo asistente del Servicio de Anatomia Patológica del INSN-San Borja

Published

2017-06-28

Issue

Section

Original Article

How to Cite

1.
Torres-Iberico R, Palomo-Luck P, Torres-Ramos G, Lipa-Chancolla R. Epidermolysis bullosa in Peru: clinical and epidemiological study of patients treated in a national reference pediatric hospital, 1993-2015. Rev Peru Med Exp Salud Publica [Internet]. 2017 Jun. 28 [cited 2024 Mar. 28];34(2):201-8. Available from: https://rpmesp.ins.gob.pe/index.php/rpmesp/article/view/2484

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