Main Article Content
Abstract
Background: Lamellar ichthyosis (LI) is a rare non-syndromic congenital ichthyosis and is autosomal recessive. LI patients are generally born with abnormalities of keratinization, then develop into lamellar scales and persist into adulthood. This study aimed to describe cases of lamellar ichthyosis and provide appropriate management to prevent worsening patient prognosis.
Case presentation: A 6-year-old girl and her younger brother, 2-year-old have been complaining of scaly skin all over their bodies since birth. Both patients were born with a history of being covered by a collodion membrane. On physical examination, thick and wide lamellar scales were seen with an erythematous base and palmoplantar hyperkeratosis. Ectropion of the eyelids, eclabium in the mouth area, and microtia in the ears were seen. The results of the dermoscopy examination showed brownish quadrilateral structures with white scales forming a lamellar pattern. Laboratory examination showed vitamin D insufficiency. Both patients were referred to the Pediatric Polyclinic regarding insufficiency and developmental complaints. Patients received therapy in the form of moisturizers, artificial eye drops, topical urea as a keratolytic, and oral vitamin D 2,000 IU per day. There was a clinical improvement after 3 months of treatment.
Conclusion: Lamellar ichthyosis is an autosomal recessive congenital ichthyosis with clinical manifestations limited to the skin. In a minority of cases, LI can be inherited in an autosomal dominant manner. Thorough management is necessary to prevent a poor prognosis in patients.
Keywords
Article Details
As our aim is to disseminate original research article, hence the publishing right is a necessary one. The publishing right is needed in order to reach the agreement between the author and publisher. As the journal is fully open access, the authors will sign an exclusive license agreement.
The authors have the right to:
- Share their article in the same ways permitted to third parties under the relevant user license.
- Retain copyright, patent, trademark and other intellectual property rights including research data.
- Proper attribution and credit for the published work.
For the open access article, the publisher is granted to the following right.
- The non-exclusive right to publish the article and grant right to others.
- For the published article, the publisher applied for the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.