RESUMEN
20-year-old patient presented with the episodes of generalized hyperkeratotic lesions with bullae since her early life, without family history. Histopathological examination by light and electron microscopes showed the characteristic features of epidermolytic hyperkeratosis. Primarily, she failed to respond to the treatment with propylene glycol. Vitamine A palmitate(A-Mulsin) per os appears to be a beneficial remedy for epidermolytic hyperkeratosis, although its availability is limited due to the side effects on a long term therapy. Repeated biopsies in the normal appearing lesions 2 months after treatment of vitarnin .A palmitate showed a substantial reduction of the horny layer on the light microscope and orderly arrangement of the tonofilaments, and properly formed keratohyaline granules on EM, but the underlying disorder of keratinization remained unchanged. Treattnent of 2 months with vitamin A was interrupted by side effects of nasal bleealing, chelitis and xerosis.