Lamellar Ichthyosis: a case study in a neonatal unit

Authors

Keywords:

ICHTHYOSIS, LAMELLAR, CONGENITAL ABNORMALITIES, KERATODERMA, PALMOPLANTAR.

Abstract

Introduction: in the newborn, numerous hereditary disorders may be evident, which can cause various aberrations of pigmentation, texture, elasticity and structural integrity of the integument. Among these pathologies are ichthyosis, from the Greek ichthys which means fish. Laminar Ichthyosis or Congenital Ichthyosiform Erythroderma (CIE) is a rare autosomal recessive genodermatosis (1/300 000 births worldwide), characterized by an erythrodermic condition, palmoplantar keratoderma (PPK), onicosis, and other disorders.

Case report: a newborn with Lamellar Ichthyosis hospitalized in the neonatal intensive care unit at Fe del Valle Ramos Gynecological Obstetric Hospital, in Manzanillo municipality, Granma province. Medical care was based on maintaining the integrity of the skin through moisturizing and continuous lubrication with emollients, temperature control, nutrition and prevention of secondary infections.

Conclusions: through the case study, it is stated that the medical appointments with other specialties for the multidisciplinary care and the implementation of the care plan were essential regarding the multidisciplinary success of the treatment. There was improvement of the skin and mucosa, prevention of infections, concluding with favorable conditions of survival and autonomy of the parents for home care.

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Author Biographies

Giselle Lucila Vazquez Gutierrez, Universidad de Ciencias Médicas de Granma. Dirección de Ciencia e Innovación Tecnológica. Granma. Cuba

Doctora en Medicina. Especialista de  Segundo  Grado en Pediatría. Profesor Asistente. Investigadora Agregada.

Gabriel Granados Perez, Hospital Ginecobstetrico Fe del Valle Ramos. Granma. Cuba

Doctor en Medicina. Especialista de Primer Grado en Neonatología

Jesús Daniel de la Rosa Santana, Universidad de Ciencias Médicas de Granma. Facultad de Ciencias Médicas Celia Sánchez Manduley. Manzanillo, Cuba.

Estudiante de 5to año de Medicina. Alumno Ayudante de Cardiología

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Published

2020-07-30

How to Cite

1.
Vazquez Gutierrez GL, Granados Perez G, de la Rosa Santana JD. Lamellar Ichthyosis: a case study in a neonatal unit. Rev Ciencias Médicas [Internet]. 2020 Jul. 30 [cited 2025 Aug. 26];24(4):e4466. Available from: https://revcmpinar.sld.cu/index.php/publicaciones/article/view/4466

Issue

Section

CASE REPORTS