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Abstract:
A 13-month-old child with lamellar ichthyosis and numerous cutaneous and systemic infections died following an abrupt episode of severe hypotension. Postmortem examination revealed white matter vacuolation in the brain stem tegmentum and cerebellar hemispheres similar to that seen in toxicity from hexachlorophene, to which our patient was not exposed. The patient had been treated with many commonly used medications, including 13-cis-retinoic acid, a relatively new drug with apparent therapeutic benefit in hyperkeratotic dermatoses. This had been given systemically during the patient's last week of life. Possible relationships between the white matter lesions and extrinsic toxic agents are discussed.
Insights
A child with lamellar ichthyosis died from hypotension. Postmortem revealed brain lesions similar to hexachlorophene toxicity, though the child received 13-cis-retinoic acid.
Area of Science:
- Dermatology
- Neurology
- Toxicology
Background:
- Lamellar ichthyosis is a severe congenital skin disorder.
- Infants with ichthyosis are prone to infections and complications.
- Systemic treatments for ichthyosis aim to manage hyperkeratosis.
Observation:
- A 13-month-old infant with lamellar ichthyosis experienced sudden hypotension and died.
- Autopsy revealed white matter vacuolation in the brainstem and cerebellum.
- These lesions resembled those seen in hexachlorophene toxicity.
Findings:
- The infant was treated with 13-cis-retinoic acid in the week prior to death.
- No exposure to hexachlorophene was identified.
- A potential link between 13-cis-retinoic acid and the observed neurological lesions is investigated.
Implications:
- This case highlights potential neurotoxicity of 13-cis-retinoic acid in infants with ichthyosis.
- Further research is needed to understand the safety profile of retinoids in pediatric dermatoses.
- Careful monitoring for neurological adverse events is crucial in patients receiving systemic retinoid therapy.