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Darier's Disease/Keratosis Follicularis
Published in Charles Theisler, Adjuvant Medical Care, 2023
Darier’s disease, also known as keratosis follicularis, is an inherited skin condition characterized by small, thin, greasy bumps that are yellow to brown in color and can emit a strong unpleasant odor. The skin lesions generally develop a brown, greasy crust and become thickened, warty (hyperkeratotic), scaly, and darkened. The lesions slowly grow bigger, eventually coming together (coalescing) to form discolored, warty plaques that may cover extensive areas of the body particularly on the trunk.1 The most common sites for blemishes are the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. The nails and mucous membranes are also affected in most cases.1
Mechanism of Action of Alitretinoin
Published in Ayse Serap Karadag, Berna Aksoy, Lawrence Charles Parish, Retinoids in Dermatology, 2019
Ömer Faruk Elmas, Necmettin Akdeniz
Darier disease is an autosomal dominant inherited dermatosis characterized by warty, dirty gray-colored papular lesions distributed in seborrheic areas. There are several studies reporting that alitretinoin is an effective option in treating patients with Darier disease (25–27). Alitretinoin may be considered as a regulator for cell proliferation, differentiation, keratinization, and immunomodulation (1).
Disorders of keratinization and other genodermatoses
Published in Ronald Marks, Richard Motley, Common Skin Diseases, 2019
Darier’s disease is an uncommon disorder that appears to be inherited as an autosomal dominant disorder, but also occurs sporadically. A characteristic feature is the appearance of groups of brownish, horny papules over the central trunk, shoulders, face and also elsewhere (Figs 18.14 and 18.15). These papules easily become irritated and/or infected and become exudative and crusted. Other features include the presence of tiny pits on the palms and a nail dystrophy in which there is a vertical ridge starting at an indentation at the free border. There is an association with serious psychiatric disorder, particularly schizophrenia in some patients.
Skin diseases of the vulva: inflammatory, erosive-ulcerating and apocrine gland diseases, zinc and vitamin deficiency, vulvodynia and vestibulodynia
Published in Journal of Obstetrics and Gynaecology, 2018
Freja Lærke Sand, Simon Francis Thomsen
Darier’s disease is an autosomal dominant chronic cutaneous disorder with variable penetrance affecting women between the ages of 20 and 30. The exact pathogenesis is unknown but the main finding is a defect in the tonofilament–desmosome complex resulting in epidermal acantholysis (Ackerman 1972; Cooper 1989). The typical sites involved are the chest, abdomen and back but a minority of women presents with a flexural variant of the disease showing greasy, crusted, yellow-brown confluent papules in the genital area (Figure 14). Secondary infection due to staphylococci or Herpes simplex virus may result in oozing and malodorous lesions.
A case of severe refractory Darier’s disease of the feet in which management with carbon dioxide (CO2) laser therapy demonstrated superior outcomes to surgical excision
Published in Journal of Cosmetic and Laser Therapy, 2020
Anna V. Dunnigan, Brogan Salence, Tess McPherson, Neil P. J. Walker
Darier’s disease, also known as Darier-White disease, is a rare autosomal dominant genodermatosis caused by mutations in the ATP2A2 gene encoding SERCA2, a sarco/endoplasmic reticulum calcium ATP-ase pump. This affects the calcium ion-mediated maturation of desmosomes and cellular adhesion proteins, resulting in weakened intracellular adhesion. The disease classically presents as multiple scaly or crusted papules. The diagnosis is frequently clinical, but may be aided by the identification of characteristic histological findings on skin biopsy.