An Update on the Cutaneous Manifestations of Darier Disease

Algit Yeshurun, Michael Ziv, Eran Cohen-Barak, Shiraz Vered, Dganit Rozenman, Muhammad Sah, Morad Khayat, Olga Polyakov, Boaz Amichai, Abraham Zlotogorski, Stavit Shalev, Roni P. Dodiuk-Gad

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)498-503
Number of pages6
JournalJournal of Cutaneous Medicine and Surgery
Volume25
Issue number5
DOIs
StatePublished - Sep 2021

Keywords

  • clinical manifestations
  • Darier disease
  • keratosis folicullaris

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