AI Article Synopsis
- The study at Instituto Nacional de Salud (INSN) analyzed clinical and epidemiological features of 93 patients diagnosed with epidermolysis bullosa (EB) from 1993 to 2015, focusing on their age, gender, and clinical forms.
- The majority of the patients were young boys, with dystrophic EB being the most common type, followed by simple, union, and Kindler syndrome.
- The research highlighted significant extrcutaneous complications, such as gastrointestinal and nutritional issues, and identified a considerable prevalence of malnutrition and anemia among the patients, with a reported mortality rate of 6.5%.
Article 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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| http://dx.doi.org/10.17843/rpmesp.2017.342.2484 | DOI Listing |
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