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vol.99 issue4REVIEW OF THE LITERATURE ON PRIMARY EXTRAMAMMARY PAGET DISEASE IN SCROTUM: A PURPOSE OF A CASECUTANEOUS MELANOMA OF THE DORSAL REGION.A CASE REPORT author indexsubject indexarticles search
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Revista argentina de dermatología

On-line version ISSN 1851-300X

Abstract

LIMA, PMO et al. CLINICAL PRESENTATION OF LINEAR DARIER’S DISEASE. A CASE REPORT. Rev. argent. dermatol. [online]. 2018, vol.99, n.4, pp.51-58. ISSN 1851-300X.

Darier's disease is an autosomal dominant genodermatosis, characterized by acatholysis and abnormal keratinization, with characteristic mucocutaneous findings, such as keratotic papules in the upper trunk and longitudinal erythronychia. There is a localized rare presentation that corresponds to a genetic mosaicism, which involves the surfaceof the skin in a linear, zosteriform, irregular or other localized arrangement, being the trunk and limbs the most affected areas.

We report amosaicism of Darier's disease, whose presentation was in a linear, unilateral pattern, following the lines of Blaschko, with localized hypertrophic formation, configuring the Linear Darier’s disease. The patient presented with multiple hypochromic planes along the right lower limb, from the thigh root to the nail matrix of the first toe. In the right inguinal region, there was confluence of the papules, forming a condylomatous, grayish and pruritic plaque. We chose to perform histopathological examination from a papule on the leg and the inguinal plate. The therapy was directed to improve the patient’s quality of life.

Keywords : Darier´s disease; mosaicism; linear; genodermatosis; keratotic papules..

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