Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering
Superficial epidermolytic ichthyosis (SEI), formerly known as ichthyosis bullosa of Siemens (IBS), is an extremely rare keratinization disorder with superficial peeling, with an estimated prevalence of 1:500,000, caused by a variety of mutations in the keratin 2E gene. The clinical features...
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| Format: | Article |
| Language: | English |
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Our Dermatology Online
2022-01-01
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| Series: | Nasza Dermatologia Online |
| Online Access: | http://www.odermatol.com/issue-in-html/2022-1-22-epidermolytic_ichthyosis/ |
| _version_ | 1797977115976531968 |
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| author | Ashwani Rana Prajul Mehta |
| author_facet | Ashwani Rana Prajul Mehta |
| author_sort | Ashwani Rana |
| collection | DOAJ |
| description | Superficial epidermolytic ichthyosis (SEI), formerly known as ichthyosis bullosa of Siemens (IBS), is an extremely rare keratinization disorder with superficial peeling, with an estimated prevalence of 1:500,000, caused by a variety of mutations in the keratin 2E gene. The clinical features include hyperkeratosis and blistering, but these are milder than in epidermolytic hyperkeratosis. The treatment is symptomatic and involves keratolytics and emollients. Herein, we report a case of SEI with the unusual absence of spontaneous blistering. |
| first_indexed | 2024-04-11T05:01:47Z |
| format | Article |
| id | doaj.art-36851dd5b9004dae85907e7dc5de669e |
| institution | Directory Open Access Journal |
| issn | 2081-9390 |
| language | English |
| last_indexed | 2024-04-11T05:01:47Z |
| publishDate | 2022-01-01 |
| publisher | Our Dermatology Online |
| record_format | Article |
| series | Nasza Dermatologia Online |
| spelling | doaj.art-36851dd5b9004dae85907e7dc5de669e2022-12-25T15:58:38ZengOur Dermatology OnlineNasza Dermatologia Online2081-93902022-01-01131899110.7241/ourd.20221.22Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blisteringAshwani RanaPrajul Mehta Superficial epidermolytic ichthyosis (SEI), formerly known as ichthyosis bullosa of Siemens (IBS), is an extremely rare keratinization disorder with superficial peeling, with an estimated prevalence of 1:500,000, caused by a variety of mutations in the keratin 2E gene. The clinical features include hyperkeratosis and blistering, but these are milder than in epidermolytic hyperkeratosis. The treatment is symptomatic and involves keratolytics and emollients. Herein, we report a case of SEI with the unusual absence of spontaneous blistering.http://www.odermatol.com/issue-in-html/2022-1-22-epidermolytic_ichthyosis/ |
| spellingShingle | Ashwani Rana Prajul Mehta Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering Nasza Dermatologia Online |
| title | Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering |
| title_full | Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering |
| title_fullStr | Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering |
| title_full_unstemmed | Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering |
| title_short | Superficial epidermolytic ichthyosis: A rare disorder with the unusual absence of blistering |
| title_sort | superficial epidermolytic ichthyosis a rare disorder with the unusual absence of blistering |
| url | http://www.odermatol.com/issue-in-html/2022-1-22-epidermolytic_ichthyosis/ |
| work_keys_str_mv | AT ashwanirana superficialepidermolyticichthyosisararedisorderwiththeunusualabsenceofblistering AT prajulmehta superficialepidermolyticichthyosisararedisorderwiththeunusualabsenceofblistering |