Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf

Darier disease (DD) is a rare genodermatoses characterized by greasy hyperkeratotic papules in seborrheic regions and nail and oral changes. Histologically, it presents as suprabasal clefts with acantholytic and dyskeratotic cells. Acrokertasosis verruciformis of Hopf (AKVH) is considered an allelic...

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Main Authors: Vikash Paudel, Manish Bhakta Pradhan, Brijesh Shrestha, Sumit Paudel
Format: Article
Language:English
Published: Wiley 2022-01-01
Series:Case Reports in Dermatological Medicine
Online Access:http://dx.doi.org/10.1155/2022/5233837
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author Vikash Paudel
Manish Bhakta Pradhan
Brijesh Shrestha
Sumit Paudel
author_facet Vikash Paudel
Manish Bhakta Pradhan
Brijesh Shrestha
Sumit Paudel
author_sort Vikash Paudel
collection DOAJ
description Darier disease (DD) is a rare genodermatoses characterized by greasy hyperkeratotic papules in seborrheic regions and nail and oral changes. Histologically, it presents as suprabasal clefts with acantholytic and dyskeratotic cells. Acrokertasosis verruciformis of Hopf (AKVH) is considered an allelic variant with clinical overlap where Church spires are seen histologically without dyskeratoses. Patients are susceptible to various viral and bacterial skin infections requiring prevention and treatment of infection. Nonspecific treatment includes patient counseling on exacerbating factors. Although there are no curative treatments for DD, topical corticosteroids and systemic retinoids may be used to control inflammation and hyperkeratosis. We are reporting a rare case with clinical and histological findings of DD with AKVH in a 17-year-old boy with keratotic papules, presented on the hands and feet, nose, and ears without mucosal involvement.
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spelling doaj-art-3f63893db3c84649bbb8cb20f8303a032025-02-03T06:12:24ZengWileyCase Reports in Dermatological Medicine2090-64712022-01-01202210.1155/2022/5233837Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of HopfVikash Paudel0Manish Bhakta Pradhan1Brijesh Shrestha2Sumit Paudel3Department of Dermatology and VenereologyDepartment of Dermatology and VenereologyDepartment of PathologyDepartment of Dermatology and VenereologyDarier disease (DD) is a rare genodermatoses characterized by greasy hyperkeratotic papules in seborrheic regions and nail and oral changes. Histologically, it presents as suprabasal clefts with acantholytic and dyskeratotic cells. Acrokertasosis verruciformis of Hopf (AKVH) is considered an allelic variant with clinical overlap where Church spires are seen histologically without dyskeratoses. Patients are susceptible to various viral and bacterial skin infections requiring prevention and treatment of infection. Nonspecific treatment includes patient counseling on exacerbating factors. Although there are no curative treatments for DD, topical corticosteroids and systemic retinoids may be used to control inflammation and hyperkeratosis. We are reporting a rare case with clinical and histological findings of DD with AKVH in a 17-year-old boy with keratotic papules, presented on the hands and feet, nose, and ears without mucosal involvement.http://dx.doi.org/10.1155/2022/5233837
spellingShingle Vikash Paudel
Manish Bhakta Pradhan
Brijesh Shrestha
Sumit Paudel
Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf
Case Reports in Dermatological Medicine
title Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf
title_full Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf
title_fullStr Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf
title_full_unstemmed Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf
title_short Clinical and Histopathological Findings in a Patient of Darier–White Disease with Acrokertasosis Verruciformis of Hopf
title_sort clinical and histopathological findings in a patient of darier white disease with acrokertasosis verruciformis of hopf
url http://dx.doi.org/10.1155/2022/5233837
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