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...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
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/ |
| Summary: | 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. |
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| ISSN: | 2081-9390 |