Abstract
Darier’s disease (DD) is an autosomal dominant disease induced by haplo-insufficiency with variable expressivity but with complete penetrance in adults. Considerable variation in severity and in clinical manifestation was found between families. Lesions are hyperkeratotic, greasy papules, which are skin colored, yellow, or brown, that may coalesce into large plaques. Pruritus occurs in 80 % of patients and may be intractable. Nails, mucous, and folds can be involved. The disease usually has a chronic relapsing course. Diagnosis must be confirmed by histology, showing focal acantholytic dyskeratosis. Sun and heat avoidance, use of sunscreens, and maintenance of personal hygiene can be helpful. Clothing should be cool cotton and not too tight.
Therapy is challenging and sometimes frustrating. It is based on topical and systemic retinoids or surgical procedures.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Further Reading
Avery HL, Hughes BR, Coley C, Cooper HL. Clinical improvement in Darier’s disease with photodynamic therapy. Australas J Dermatol. 2010;51(1):32–5.
Bach DR, Moggi F, Müller TJ, Seifritz E, Strik WK, Wirtz G. Possible genetic link between Darier’s disease and depression. Review of the literature and case history. Nervenarzt. 2007;78(1):81–4.
Bhat RM, Ullal KR, Pinto AC, Sukumar D. Darier-White disease in siblings responding to isotretinoin. Indian Dermatol Online J. 2010;1(1):18–20.
Brazzelli V, Prestinari F, Barbagallo T, Vassallo C, Agozzino M, Borroni G. Linear Darier’s disease successfully treated with 0.1% tazarotene gel “short-contact” therapy. Eur J Dermatol. 2006;16(1):59–61.
Brown VL, Kelly SE, Burge SM, Walker NP. Extensive recalcitrant Darier disease successfully treated with laser ablation. Br J Dermatol. 2010;162(1):227–9.
Burge SM, Wilkinson JD. Darier-White disease: a review of the clinical features in 163 patients. Am Acad Dermatol. 1992;27(1):40–50.
Dodiuk-Gad R, Cohen-Barak E, Ziv M, Shani-Adir A, Amichai B, Zlotogorski A, Shalev S, Rozenman D. Health-related quality of life among Darier’s disease patients. J Eur Acad Dermatol Venereol. 2013;27(1):51–6.
Eimer L, Lagodin C, Bonavia P, Stringa M, Rébora I, Anaya J. Darier-White disease treated with oral isotretinoin. Arch Argent Pediatr. 2011;109(4):e63–6 [Article in Spanish].
Exadaktylou D, Kurwa HA, Calonje E, Barlow RJ. Treatment of Darier’s disease with photodynamic therapy. Br J Dermatol. 2003;149(3):606–10.
Gordon-Smith K, Jones LA, Burge SM, Munro CS, Tavadia S, Craddock N. The neuropsychiatric phenotype in Darier disease. Br J Dermatol. 2010;163(3):515–22.
Hovnanian A. Acantholytic disorders of the skin: Darier-White Disease, Acrokeratosis verruciformis, Grover disease and Hailey-Hailey Disease. In Wolff K, GoldsmithLA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ (eds.)., Fitzpatrick’s Dermatology in general medicine. McGraw-Hill Companies, USA, 2008, p. 432–42.
Hulatt L, Burge S. Darier’s disease: hopes and challenges. J R Soc Med. 2003;96(9):439–41.
Judge MR, McLean WHI, Munro GS. Disorders of Keratinization. In: Rook’s Textbook of Dermatology. Burns T, Breathnach S, Cox N, Griffiths C (eds), 2010, Blackwell Publishing Ltd.
Katz TM, Firoz BF, Goldberg LH, Friedman PM. Treatment of Darier’s disease using a 1,550-nm erbium-doped fiber laser. Dermatol Surg. 2010;36(1):142–6.
Pérez-Carmona L, Fleta-Asín B, Moreno-García-Del-Real C, Jaén-Olasolo P. Successful treatment of Darier’s disease with topical pimecrolimus. Eur J Dermatol. 2011;21(2):301–2.
Roos S, Karsai S, Ockenfel HM, Raulin C. Successful treatment of Darier disease with the flashlamp-pumped pulsed-dye laser. Arch Dermatol. 2008;144(8):1073–5.
van’t Westeinde SC, Sanders CJ, van Weelden H. Photodynamic therapy in a patient with Darier’s disease. J Eur Acad Dermatol Venereol. 2006;20(7):870–2.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Bardazzi, F., Odorici, G., Balestri, R. (2015). Darier’s Disease. In: Katsambas, A.D., Lotti, T.M., Dessinioti, C., D’Erme, A.M. (eds) European Handbook of Dermatological Treatments. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45139-7_18
Download citation
DOI: https://doi.org/10.1007/978-3-662-45139-7_18
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-662-45138-0
Online ISBN: 978-3-662-45139-7
eBook Packages: MedicineMedicine (R0)