An Update on the Cutaneous Manifestations of Darier Disease. Issue 5 (September 2021)

• Record Type: Journal Article
• Authors: Yeshurun, Algit; Ziv, Michael; Cohen-Barak, Eran; Vered, Shiraz; Rozenman, Dganit; Sah, Muhammad; Khayat, Morad; Polyakov, Olga; Amichai, Boaz; Zlotogorski, Abraham; Shalev, Stavit; Dodiuk-Gad, Roni P.
• Is Part Of: Journal of cutaneous medicine and surgery. Volume 25:Issue 5(2021)
• Page Start: 498
• Page End: 503
• Publication Date: 2021-09
• Subjects: Darier disease -- clinical manifestations -- keratosis folicullaris; Dermatology -- Periodicals; Skin -- Diseases -- Treatment -- Periodicals; Skin -- Surgery -- Periodicals; Dermatology -- Canada -- Periodicals; Skin -- Diseases -- Treatment -- Canada -- Periodicals; Skin -- Surgery -- Canada -- Periodicals; 616.5005
• DOI: 10.1177/1203475421999331
• Languages: English
• ISSNs: 1203-4754

Abstract

Background: Knowledge about the clinical features of Darier disease, an orphan autosomal-dominant genetic disorder, is sparse and has been evaluated only in few studies. Objectives: To investigate the clinical features of a large group of patients with Darier disease, and to explore for associations between disease characteristics and severity of the disease. Methods: Seventy-six individuals with Darier disease were evaluated utilizing a structured questionnaire-based interview, a physical examination, and a retrospective assessment of their medical records. Results: The most frequent locations of lesions were hands (99%) and fingernails (93%). Wart-like lesions on the hands were more visible after soaking them in water for 5 minutes, we therefore named this phenomenon the "wet hand sign". Oral involvement was found in 43% of patients, while 48% of women and 16% of men showed genital lesions. Patients with severe Darier disease had a tenfold greater risk of developing genital lesions than those with mild disease ( P = .01). Most patients (88%) in our study exhibited a combination of the four types of the disease patterns of distribution (flexural, seborrheic, nevoid, and acral). Conclusions: Documentation of disease on the hands and fingernails provides a highly sensitive means to aid in the diagnosis of Darier disease. It is important to evaluate mucosal lesions including genital and oral mucosa.