A Rare Clinical Presentation of Darier’s Disease
Darier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic a...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | Case Reports in Dermatological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2013/419797 |
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| author | Mybera Ferizi Antigona Begolli-Gerqari Bostjan Luzar Fisnik Kurshumliu Mergita Ferizi |
| author_facet | Mybera Ferizi Antigona Begolli-Gerqari Bostjan Luzar Fisnik Kurshumliu Mergita Ferizi |
| author_sort | Mybera Ferizi |
| collection | DOAJ |
| description | Darier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions are presented as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains”. This paper reports a case of a 53-year-old woman that was admitted to our clinic with more than 10-year history of keratotic papules, presented on the hands and feet, nose, ears, genitalia, and whitish lesions on palatal mucosae. |
| format | Article |
| id | doaj-art-c0fa2549a4014c9d8f32655185ed8682 |
| institution | Kabale University |
| issn | 2090-6463 2090-6471 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Dermatological Medicine |
| spelling | doaj-art-c0fa2549a4014c9d8f32655185ed86822025-02-03T06:48:28ZengWileyCase Reports in Dermatological Medicine2090-64632090-64712013-01-01201310.1155/2013/419797419797A Rare Clinical Presentation of Darier’s DiseaseMybera Ferizi0Antigona Begolli-Gerqari1Bostjan Luzar2Fisnik Kurshumliu3Mergita Ferizi4Department of Dermatology, University Clinical Center, 10000 Pristina, KosovoDepartment of Dermatology, University Clinical Center, 10000 Pristina, KosovoInstitute of Pathology, University of Ljubljana, 1000 Ljubljana, SloveniaInstitute of Pathology, University Clinical Center, 10000 Pristina, KosovoMedical Faculty, University of Pristina, 10000 Pristina, KosovoDarier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions are presented as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains”. This paper reports a case of a 53-year-old woman that was admitted to our clinic with more than 10-year history of keratotic papules, presented on the hands and feet, nose, ears, genitalia, and whitish lesions on palatal mucosae.http://dx.doi.org/10.1155/2013/419797 |
| spellingShingle | Mybera Ferizi Antigona Begolli-Gerqari Bostjan Luzar Fisnik Kurshumliu Mergita Ferizi A Rare Clinical Presentation of Darier’s Disease Case Reports in Dermatological Medicine |
| title | A Rare Clinical Presentation of Darier’s Disease |
| title_full | A Rare Clinical Presentation of Darier’s Disease |
| title_fullStr | A Rare Clinical Presentation of Darier’s Disease |
| title_full_unstemmed | A Rare Clinical Presentation of Darier’s Disease |
| title_short | A Rare Clinical Presentation of Darier’s Disease |
| title_sort | rare clinical presentation of darier s disease |
| url | http://dx.doi.org/10.1155/2013/419797 |
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