Tous Romero Fátima, Burillo Martinez Sara, Raya-Morales Carlos, Gargallo-Moneva Vanessa, Maroñas-Jiménez Lidia
Department of Dermatology, Hospital 12 de Octubre, Madrid, Spain.
Dermatol Online J. 2016 Jun 15;22(6):13030/qt436496ns.
Darier disease (DD) is an autosomal dominant genodermatosis characterized by multiple keratotic and crusted papules over seborrheic areas, along with a variable involvement of oral mucosa, palmoplantar region, and nails. Segmental subtypes (type 1 and 2) are uncommon clinically limited forms of DD that usually present at middle age with few cutaneous lesions following Blaschko´s lines. We report a case of extensive multi segmental DD type 1 that developed in an elderly man, an unusual clinical onset of DD that dermatologists should bear in mind.
Darier病(DD)是一种常染色体显性遗传的皮肤疾病,其特征为脂溢性区域出现多个角化性和结痂性丘疹,同时口腔黏膜、掌跖区域和指甲也有不同程度受累。节段型亚型(1型和2型)是临床上少见的DD局限性形式,通常在中年发病,沿Blaschko线分布有少量皮肤损害。我们报告一例老年男性发生的广泛多节段1型DD病例,这是DD一种不寻常的临床发病情况,皮肤科医生应予以关注。