title: Vacuolar myelinopathy of the brain and lamellar ichthyosis.
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.
authors.forename: N
authors.initials: N
authors.lastname: Sadick
authors.forename: P E
authors.initials: PE
authors.lastname: Sternberg
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use / Q000633:toxicity; D000890:Anti-Infective Agents / Q000627:therapeutic use / Q000633:toxicity; D001424:Bacterial Infections / Q000188:drug therapy; D001933:Brain Stem / Q000473:pathology; D002493:Central Nervous System Diseases / Q000139:chemically induced* / Q000473:pathology; D002552:Cerebral Ventricles / Q000473:pathology; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007057:Ichthyosis / Q000188:drug therapy* / Q000473:pathology; D007223:Infant; D015474:Isotretinoin; D009181:Mycoses / Q000188:drug therapy; D009186:Myelin Sheath / Q000473:pathology; D012874:Skin Diseases, Infectious / Q000188:drug therapy; D014212:Tretinoin / Q000627:therapeutic use / Q000633:toxicity*; D014617:Vacuoles / Q000648:ultrastructure
chemical_list: D000900:Anti-Bacterial Agents; D000890:Anti-Infective Agents; D014212:Tretinoin; D015474:Isotretinoin