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INTRODUCTION
Darier disease (DD) also called ‘Darier-White disease’ is a rare slowly progressive genetic skin disorder characterized by multiple keratotic papules. There is a loss of adhesion and an inadequate binding between epidermal cells caused by mutations in the gene mapped onto a single locus on chromosome 12q23-q24, which encodes a sarco/endoplasmic reticulum calcium ATPase pump (SERCA2) [1]. This gene is responsible for calcium transport into cells. Penetrance is high but expressivity is variable so, if a patient is greatly affected it does not necessarily mean that other members will get this severe disease [2,3]. The gene is expressed also in the brain [4]. DD begins in childhood and affects men and women the same. Clinically its classical form shows yellow-brown hyperkeratotic rough papules that coalesce into warty plaques on central trunk, scalp, forehead, and flexures, in a way simulating seborrhea. Abnormal keratinocyte adhesion and aberrant epidermal keratinization are the primary histologic features also with suprabasilar splitting and villus-like structures in the dermis. Dyskeratosis is demonstrated by corps-rounds and grains with overlying hyperkeratosis.
CASE REPORT
A 59-year-old metalworker male attended our clinic with a right-sided skin eruption, which he noted when he was 16 years old. No family members had similar conditions. The eruption was pruritic and returned almost every summer. Reddish-brown dirty-coloured keratotic papules with greasy appearance spread in a swirling distribution along the Blaschko lines beginning on the anterior surface of the right thigh, the lateral surface of the right knee and the anterior e lateral surfaces of the right leg (Fig. 1 [See PDF]). Also, on the lateral and posterior surfaces of the right arm, from the deltoid region to a few inches above the elbow (Fig. 2 [See PDF]). It was often...