Basal Cell Carcinoma in Type 2 Segmental Darier's Disease

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...

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Main Authors: Lynne Robertson, Maxwell B. Sauder
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Journal of Skin Cancer
Online Access:http://dx.doi.org/10.1155/2012/839561
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author Lynne Robertson
Maxwell B. Sauder
author_facet Lynne Robertson
Maxwell B. Sauder
author_sort Lynne Robertson
collection DOAJ
description 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.
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spelling doaj-art-0fd4040359994af7a2dfcf59bd3b255d2025-02-03T05:44:28ZengWileyJournal of Skin Cancer2090-29052090-29132012-01-01201210.1155/2012/839561839561Basal Cell Carcinoma in Type 2 Segmental Darier's DiseaseLynne Robertson0Maxwell B. Sauder1Division of Dermatology, Department of Medicine, University of Calgary, Calgary, AB, CanadaDivision of Dermatology, Department of Medicine, University of Ottawa, Ottawa, ON, CanadaBackground. 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.http://dx.doi.org/10.1155/2012/839561
spellingShingle Lynne Robertson
Maxwell B. Sauder
Basal Cell Carcinoma in Type 2 Segmental Darier's Disease
Journal of Skin Cancer
title Basal Cell Carcinoma in Type 2 Segmental Darier's Disease
title_full Basal Cell Carcinoma in Type 2 Segmental Darier's Disease
title_fullStr Basal Cell Carcinoma in Type 2 Segmental Darier's Disease
title_full_unstemmed Basal Cell Carcinoma in Type 2 Segmental Darier's Disease
title_short Basal Cell Carcinoma in Type 2 Segmental Darier's Disease
title_sort basal cell carcinoma in type 2 segmental darier s disease
url http://dx.doi.org/10.1155/2012/839561
work_keys_str_mv AT lynnerobertson basalcellcarcinomaintype2segmentaldariersdisease
AT maxwellbsauder basalcellcarcinomaintype2segmentaldariersdisease