Darier-white disease with variable penetrance

Author: 
Navein R K., Srivenkateswaran K., Sivasubramanian V., Sivakumar.S and Kirandeepkaur Chadha

Darier disease is an autosomal dominant genodermatosis with incomplete penetrance and variable expressivity. Estimated incidence is 4 per million per 10 years. Men and women are equally affected. Characteristic mucocutaneous findings such as hyperkeratotic papules preferentially in seborrheic distribution areas and longitudinal erythronychia with V shaped notches at distal edge of nail and whitish oral mucosal papules in some people. Histological features includes acantholysis and dyskeratosis (corp ronds and grains)

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DOI: 
http://dx.doi.org/10.24327/ijcar.2019.16880.3138
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