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Cholesteatoma in a child with congenital ichthyosiform erythroderma
M Bartolomé1, A Torrelo, E Molina
1Department of Pediatric Otolaryngology, Hospital del Niño Jesús, Madrid, Spain.
Insights
Non-bullous congenital ichthyosiform erythroderma (NBCIE) is linked to childhood cholesteatoma. Chronic scaling skin conditions increase the risk of ear infections and cholesteatoma development.
Area of Science:
- Dermatology
- Otolaryngology
- Pediatrics
Background:
- Non-bullous congenital ichthyosiform erythroderma (NBCIE) is a severe genetic skin disorder.
- Patients with ichthyosis often experience complications affecting various organ systems.
- Chronic ear conditions are common in children with ichthyosis.
Observation:
- A 7-year-old girl with NBCIE, treated with retinoids, presented with continuous ear scaling and recurrent ear infections.
- The patient experienced chronic adenoiditis, tubaric dysfunction, and intermittent otitis media.
- A cholesteatoma developed in the patient's left ear.
Findings:
- Chronic scaling disorders, including NBCIE, are associated with an increased risk of cholesteatoma in children.
- The continuous desquamation of the external ear canal may predispose to infections and cholesteatoma formation.
- Early recognition and management of ear complications in ichthyosis patients are crucial.
Implications:
- This case highlights the potential link between congenital ichthyosis and pediatric cholesteatoma.
- Clinicians should be vigilant for otological manifestations in children with chronic scaling skin disorders.
- Further research is warranted to elucidate the precise mechanisms and management strategies.
Abstract:
A 7-year-old girl had non-bullous congenital ichthyosiform erythroderma (NBCIE) in treatment with retinoids. She also suffered continuous desquamation of the external ears, recurrent external otitis, chronic adenoiditis, tubaric dysfunction and intermittent episodes of otitis media and developed a cholesteatoma in her left ear. Chronic scaling disorders, such as NBCIE and other ichthyosis, can be entities at risk for the development of cholesteatoma in childhood.