Three members of a family with severe epidermolytic hyperkeratosis were successfully treated with topical tretinoin therapy. During hospitalization a controlled paired-comparison study was performed treating one half of each patient with retinoic acid and the other half with the appropriate placebo. The retinoic acid was evaluated at concentrations 0.05% and 0.1% and in both an alcohol-polyethylene glycol solution as well as in a cream base. Special studies, using the scanning electron microscope, revealed basic alterations in the ultrastructure of diseased skin which help to explain the repeated pyodermas, persistent malodor, and lack, of a physiologically effective stratum corneum with the disorder. While the ineffective barrier function permitted systemic effects from topical corticosteroid applications in one patient, no adverse reactions were seen with prolonged, full-body tretinoin therapy over a period of eight months. Significant therapeutic results were recorded photographically.
Schorr WF, Papa CM. Epidermolytic HyperkeratosisEffect of Tretinoin Therapy on the Clinical Course and the Basic Defects in the Stratum Corneum. Arch Dermatol. 1973;107(4):556-562. doi:10.1001/archderm.1973.01620190032008