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Revista argentina de dermatología

On-line version ISSN 1851-300X

Abstract

URIBE, ML; HURTADO, JH; REGAZZI, JC  and  KAWA KAC, B. Darier's disease treated with oral acitretin. Rev. argent. dermatol. [online]. 2013, vol.94, n.4, pp.25-26. ISSN 1851-300X.

Darier's disease (DD) described by Darier and White in 1889 is an autosomic dominant disorder of keratinization, caused by a mutation of gen ATP2A2, located in the chromosome 12 encoding for calcium ATPase pump of sarcoplasmic reticulum type 2 (SERCA-2) leading to abnormal keratinization (dyskeratosis) and loss of intercellular adhesion of keratinocytes (acantholysis), with the consequent formation of suprabasal clefts. Characterized for abnormal keratinization in the epidermis, nails and mucosae. We present the case of a 21-year-old, male patient, with clinically and histologically confirmed (DD) treated with oral acitretin 30 mg for day with good response. We recommend an optional therapeutic treatment for chronic, itchy, recalcitrant lesions with oral retinoids.

Keywords : Darier's disease; Dyskeratosis; Therapy; Oral retinoids; Oral acitretin.

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