title: Ichthyosiform dermatosis with superficial blister formation and peeling: evidence for a desmosomal anomaly and altered epidermal vitamin A metabolism.
abstract: We describe a man with generalized congenital ichthyosiform dermatosis, severe cheilitis, and palmar and plantar hyperkeratosis with superficial blistering. Low-dose acitretin therapy induced areas of peeling skin, similar to that seen in the peeling skin syndrome. Histologically, the skin was moderately hyperkeratotic and the palmar blisters were subcorneal. Electron microscopy revealed that the splitting occurred within the desmosomal plaque. Ultrastructural and biochemical investigations indicated epidermal hypervitaminosis A, probably related to alteration of epidermal retinoic acid metabolism. This disease is proposed as a hitherto unreported variant of the peeling skin syndrome.
authors.affiliation: Department of Dermatology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
authors.forename: B
authors.initials: B
authors.lastname: Mevorah
authors.forename: D
authors.initials: D
authors.lastname: Salomon
authors.forename: G
authors.initials: G
authors.lastname: Siegenthaler
authors.forename: D
authors.initials: D
authors.lastname: Hohl
authors.forename: M L
authors.initials: ML
authors.lastname: Meier
authors.forename: J H
authors.initials: JH
authors.lastname: Saurat
authors.forename: E
authors.initials: E
authors.lastname: Frenk
mesh_terms: D000293:Adolescent; D002613:Cheilitis / Q000209:etiology; D003896:Desmosomes / Q000648:ultrastructure; D004817:Epidermis / Q000378:metabolism; D006801:Humans; D007057:Ichthyosis / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D007645:Keratoderma, Palmoplantar / Q000209:etiology; D008297:Male; D018168:Receptors, Retinoic Acid / Q000378:metabolism; D012867:Skin / Q000473:pathology*; D014801:Vitamin A / Q000378:metabolism*
chemical_list: D018168:Receptors, Retinoic Acid; C082927:retinoic acid binding protein I, cellular; C082928:retinoic acid binding protein II, cellular; D014801:Vitamin A