China
Africa
Explore chapters and articles related to this topic
Retinoids in Keratinization Disorders
Published in Ayse Serap Karadag, Berna Aksoy, Lawrence Charles Parish, Retinoids in Dermatology, 2019
Sunblock creams are recommended to prevent sunlight-related exacerbations. Avoiding physical trauma, use of cyclosporine, systemic and topical steroids or vitamin D3 ointment, systemic and topical antibiotic or antifungal therapy to suppress bacterial and fungal infections, topical vitamin A derivatives including tazarotene, isotretinoin, and adapalene or systemic retinoids such as isotretinoin, acitretin, or alitretinoin are also recommended. Alitretinoin should be considered in young women with severe or extensive Darier disease which may be recalcitrant to isotretinoin or other therapies as pan-agonist retinoid. All treatments may be modified according to exacerbations of Darier disease. Severe and complicated patients can be successfully treated by long-term low-dose systemic retinoids like acitretin. Only topical and anti-infective treatments are recommended for patients with localized lesions. For refractory proliferative lesions, botulinum toxin injections, surgical dissection using an yttrium–aluminum–garnet laser are sometimes performed (Figure 24.3) (89,90).
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.
Nail in dermatological diseases
Published in Archana Singal, Shekhar Neema, Piyush Kumar, Nail Disorders, 2019
Piyush Kumar, Niharika Ranjan Lal
All parts of the nail unit may be affected histologically. The nail bed findings, however, are an exception, revealing the absence of suprabasilar clefts, the presence of multinucleated epithelial giant cells, and the absence of an inflammatory infiltrate. These characteristic changes lead to the diagnosis in rare cases where Darier’s disease is limited to the nails. Nail bed epithelium may be hyperplastic and exhibit subungual parakeratosis. Oral retinoids may improve keratotic papules of the proximal nail fold but other nail changes are not improved by treatment.3
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
Topical and oral therapies are usually sufficient for the management of Darier’s disease. The mainstay of oral therapies are the oral retinoids (Acitretin or Isotretinoin). However, these can have significant side effects including dry skin and mucosal surfaces, teratogenic effects and neutropenia. Furthermore, oral retinoids may not provide adequate control in severe cases of the disease. In those patients where medical therapies have failed or are unsuitable for other reasons, other options may include the use of CO2 laser therapy and surgical excision. There are a number of previous reports of the successful use of CO2 laser therapy for severe Darier’s disease, with good therapeutic outcomes achieved in all patients who had received treatment (1–5). Avoidance of triggers including mechanical trauma, sweat and excessive UV exposure may be additionally important to reduce recurrence (5). The majority of reports of surgical excision are for hypertrophic Darier’s disease, with good outcomes reported up to 3 years (6,7). Surgical excision with full thickness skin grafting has also been successfully used to treated severe Darier’s disease affecting the nail complexes of the hand (8). For disease affecting intertriginous areas (the web spaces of toes and fingers), where skin surfaces frequently rub together, or weight- bearing skin such as on the feet, long term success with surgical excision and skin grafting may be more difficult to achieve (8). There are no previous comparative studies.
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.