Darier's disease: Clinical and demographic features of nine cases
Journal Title: Türkderm - Deri Hastalıkları ve Frengi Arşivi - Year 2018, Vol 52, Issue 2
Abstract
Background and Design: Darier’s disease is a genetic disorder of keratinization with autosomal dominant inheritance. In the article presented here, demographical, clinical and histopathological findings and treatment outcome of Darier’s disease are discussed with 9 cases. Materials and Methods: We performed a retrospective study of all the patients diagnosed with Darier’s disease at the Department of Dermatology of Istanbul Training and Research Hospital, between 2008 and 2016. Results: During the observation period, we identified 9 patients with Darier’s disease; 6 males and 3 females with a mean age of 32.5 years. Two cases were from the same family (mother and child) and no family history was found in the other seven patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the head-neck and trunk. Four patients had hand involvement, five patients had nail lesions. Conclusion: Darier’s disease is a rare genodermatosis and should be considered in the differential diagnosis of dermatoses with keratotic papular lesions.
Authors and Affiliations
Sevil Savaş, Ayşe Esra Koku Aksu, Ebru Sarıkaya, Cem Leblebici, Mehmet Salih Gürel
Keywords
Related Articles
- EP ID EP364746
- DOI 10.4274/turkderm.59002
- Views 96
- Downloads 0
Effect of pulsed dye laser therapy on dermatology quality of life in patients with rosacea
Background and Design: Rosacea is a skin disease characterized by erythema, telangiectasia, inflammatory papulopustular eruption and flashing, preferentially affecting the convexities of the face. Among the various treat...
Evaluation of ischemia-modified albumin levels in acne vulgaris patients
Background and Design: Acne vulgaris (AV) is one of the most common dermatological diseases seen in late childhood and adolescence. The purpose of this study is to evaluate ischemia-modified albumin (IMA) levels in AV, a...
Comorbidities in psoriasis: The recognition of psoriasis as a systemic disease and current management
Psoriasis, with a worldwide prevalence of 2-3%, is now assumed as a systemic chronic inflammatory disease accompanied by comorbidities while it was accepted as a disease limited only to the skin in the past. There are se...
Evaluation of demographic and clinical characteristics of 166 patients with herpes zoster in the Kırşehir region
Background and Design: Herpes zoster (HZ) occurs by reactivation of the latent varicella zoster virus at dorsal root ganglia. In the literature, there are studies on socio-demographic and clinical characteristics of pati...
Mucocutaneous findings in pediatric patients with bone marrow transplantation
Background and Design: Bone marrow transplantation (BMT) is performed for many malign and non-malign diseases in pediatric patients. Cutaneous signs in transplant patients are usually encountered in the presence of under...