Ichthyosiform dermatosis with superficial blister formation and peeling: evidence for a desmosomal anomaly and altered epidermal vitamin A metabolism

Détails

ID Serval
serval:BIB_93A60255E56B
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Ichthyosiform dermatosis with superficial blister formation and peeling: evidence for a desmosomal anomaly and altered epidermal vitamin A metabolism
Périodique
Journal of the American Academy of Dermatology
Auteur(s)
Mevorah  B., Salomon  D., Siegenthaler  G., Hohl  D., Meier  M. L., Saurat  J. H., Frenk  E.
ISSN
0190-9622 (Print)
Statut éditorial
Publié
Date de publication
02/1996
Volume
34
Numéro
2 Pt 2
Pages
379-85
Notes
Case Reports
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Feb
Résumé
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.
Mots-clé
Adolescent Cheilitis/etiology Desmosomes/ultrastructure Epidermis/metabolism Humans Ichthyosis/classification/metabolism/*pathology Keratoderma, Palmoplantar/etiology Male Receptors, Retinoic Acid/metabolism Skin/*pathology Vitamin A/*metabolism
Pubmed
Web of science
Création de la notice
25/01/2008 17:36
Dernière modification de la notice
03/03/2018 19:35
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