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Journal of Skin Cancer
Volume 2012, Article ID 839561, 4 pages
Case Report

Basal Cell Carcinoma in Type 2 Segmental Darier's Disease

1Division of Dermatology, Department of Medicine, University of Calgary, Calgary, AB, Canada
2Division of Dermatology, Department of Medicine, University of Ottawa, Ottawa, ON, Canada

Received 11 March 2011; Revised 17 May 2011; Accepted 17 May 2011

Academic Editor: Daniela Massi

Copyright © 2012 Lynne Robertson and Maxwell B. Sauder. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Background. Darier's disease (DD), also known as Keratosis Follicularis or Darier-White disease, is a rare disorder of keratinization. DD can present as a generalized autosomal dominant condition as well as a localized or segmental postzygotic condition (Vázquez et al., 2002). Clinical features of DD include greasy, warty papules and plaques on seborrheic areas, dystrophic nails, palmo-plantar pits, and papules on the dorsum of the hands and feet. Objective. We report a case of basal cell carcinoma developing in a patient with type 2 segmental DD. Conclusion. According to the current literature, Type 2 segmental disease is a rare presentation of Darier's disease with only 8 previous cases reported to date. In addition, nonmelanoma skin cancer (NMSC) arising from DD is rarely reported; however, there may be an association between DD and risk of carcinogenesis.