Segmental Darier's disease: a presentation of difficult diagnosis
AbstractDarier's disease is a rare autosomal dominant genodermatosis. It has an estimated prevalence of 1 in 55,000 to 100,000 individuals, regardless of gender. It is characterized by multiple keratotic papules on the seborrheic areas of the trunk, scalp, forehead and flexures, and the clinica...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Dermatologia
2015-06-01
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| Series: | Anais Brasileiros de Dermatologia |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962015000700062&tlng=en |
| _version_ | 1818951260425945088 |
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| author | Paula Mota Medeiros Natália Ribeiro de Magalhães Alves Jeniffer Muñoz Trujillo Cássia Camarinha da Silva Paula Carolina Pessanha de Faria Roberto Souto da Silva |
| author_facet | Paula Mota Medeiros Natália Ribeiro de Magalhães Alves Jeniffer Muñoz Trujillo Cássia Camarinha da Silva Paula Carolina Pessanha de Faria Roberto Souto da Silva |
| author_sort | Paula Mota Medeiros |
| collection | DOAJ |
| description | AbstractDarier's disease is a rare autosomal dominant genodermatosis. It has an estimated prevalence of 1 in 55,000 to 100,000 individuals, regardless of gender. It is characterized by multiple keratotic papules on the seborrheic areas of the trunk, scalp, forehead and flexures, and the clinical picture is worsened by heat, sun exposure, perspiration and mechanical trauma. Histopathology observed loss of epithelial adhesion and abnormal keratinization. About 10% of cases present in the localized form of the disease. We report a case of segmental Darier's Disease Type I and discuss the main characteristics of this condition. |
| first_indexed | 2024-12-20T09:31:39Z |
| format | Article |
| id | doaj.art-b42ae389baae432bb66c8bd2e9a7779b |
| institution | Directory Open Access Journal |
| issn | 0365-0596 |
| language | English |
| last_indexed | 2024-12-20T09:31:39Z |
| publishDate | 2015-06-01 |
| publisher | Sociedade Brasileira de Dermatologia |
| record_format | Article |
| series | Anais Brasileiros de Dermatologia |
| spelling | doaj.art-b42ae389baae432bb66c8bd2e9a7779b2022-12-21T19:45:05ZengSociedade Brasileira de DermatologiaAnais Brasileiros de Dermatologia0365-05962015-06-01903 suppl 1626510.1590/abd1806-4841.20153581Segmental Darier's disease: a presentation of difficult diagnosisPaula Mota MedeirosNatália Ribeiro de Magalhães AlvesJeniffer Muñoz TrujilloCássia Camarinha da SilvaPaula Carolina Pessanha de FariaRoberto Souto da SilvaAbstractDarier's disease is a rare autosomal dominant genodermatosis. It has an estimated prevalence of 1 in 55,000 to 100,000 individuals, regardless of gender. It is characterized by multiple keratotic papules on the seborrheic areas of the trunk, scalp, forehead and flexures, and the clinical picture is worsened by heat, sun exposure, perspiration and mechanical trauma. Histopathology observed loss of epithelial adhesion and abnormal keratinization. About 10% of cases present in the localized form of the disease. We report a case of segmental Darier's Disease Type I and discuss the main characteristics of this condition.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962015000700062&tlng=enDarier's DiseaseMosaicismSkin diseasesSkin diseases, genetic |
| spellingShingle | Paula Mota Medeiros Natália Ribeiro de Magalhães Alves Jeniffer Muñoz Trujillo Cássia Camarinha da Silva Paula Carolina Pessanha de Faria Roberto Souto da Silva Segmental Darier's disease: a presentation of difficult diagnosis Anais Brasileiros de Dermatologia Darier's Disease Mosaicism Skin diseases Skin diseases, genetic |
| title | Segmental Darier's disease: a presentation of difficult diagnosis |
| title_full | Segmental Darier's disease: a presentation of difficult diagnosis |
| title_fullStr | Segmental Darier's disease: a presentation of difficult diagnosis |
| title_full_unstemmed | Segmental Darier's disease: a presentation of difficult diagnosis |
| title_short | Segmental Darier's disease: a presentation of difficult diagnosis |
| title_sort | segmental darier s disease a presentation of difficult diagnosis |
| topic | Darier's Disease Mosaicism Skin diseases Skin diseases, genetic |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962015000700062&tlng=en |
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