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

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 moder...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 1996-02, Vol.34 (2), p.379-385
Main Authors: Mevorah, B., Salomon, D., Siegenthaler, G., Hohl, D., Meier, M.L., Saurat, J.H., Frenk, E.
Format: Article
Language:English
Subjects:
Adolescent
Cheilitis - etiology
Desmosomes - ultrastructure
Epidermis - metabolism
Humans
Ichthyosis - classification
Ichthyosis - metabolism
Ichthyosis - pathology
Keratoderma, Palmoplantar - etiology
Male
Receptors, Retinoic Acid - metabolism
Skin - pathology
Vitamin A - metabolism
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Summary: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 occured 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.
ISSN:0190-9622
1097-6787
DOI:10.1016/S0190-9622(07)80013-2