Diagnosis and management of vulvar Darier disease: A case report

• Published in: Case reports in women's health 2023-09, Vol.39, p.e00545, Article e00545
• Main Authors: Evruke, Ipek, Ugurlucan, Funda Gungor, Erdem, Begum Yeni, Sari, Sule Ozturk
• Format: Article
• Language: English
• Subjects: Acantholysis; Darier disease; Retinoids; Steroids; Vulva
• ISSN: 2214-9112; 2214-9112
• DOI: 10.1016/j.crwh.2023.e00545

Darier disease is an autosomal dominant disorder with hyperkeratotic papules affecting primarily seborrheic areas of the upper chest, back, forehead, scalp, nasolabial folds, ears, and, less frequently, the oral mucosa. A typical eruption consists of keratotic and crusted skin-colored papules and plaques. Pruritus occurs in 80% of patients, and pain is rare. Lesions can be triggered by exposure to ultraviolet light, heat, or stress. Secondary infections of the lesions are a common complication. A definitive diagnosis is obtained by a biopsy showing histological features such as acantholysis, suprabasal clefts, and “corps rond and grains”. Here we present a 37-year-old woman admitted to the gynecology department with pruritic lesions she had noticed on her vulva and perineum for three months. A vulvar biopsy led to the diagnosis of Darier disease. She was referred to the dermatology department and treated with oral acitretin since systemic retinoids are offered as the first-line treatment of the disease. •Darier's disease is a rare genodermatosis, usually transmitted in an autosomal dominant pattern.•ATP2A2 gene mutations are known to be the cause of the condition.•The triggering factors include stress, exposure to ultraviolet light, heat or infection.•A biopsy of the affected area is necessary to confirm the diagnosis.•Treatment aims at symptomatic relief and an improvement in the patient's quality of life.