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Lamelar Ichthyosis
ISSN: 2582 - 6751
Publisher: author
Lamelar Ichthyosis
Indexed in
Medical Sciences
ARTICLE-FACTOR
1.3
Article Basics Score: 3
Article Transparency Score: 2
Article Operation Score: 2
Article Articles Score: 2
Article Accessibility Score: 3
SUBMIT PAPER ASK QUESTION
International Category Code (ICC):
ICC-1702
Publisher: Athenaeum Scientific Publishers
International Journal Address (IAA):
IAA.ZONE/2582117776751
eISSN
:
2582 - 6751
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Abstract
Background: Lamellar Ichthyosis (LI) is a rare hereditary autosomal recessive disease with a defect in chromosome 14q11 which generates defect of transglutaminase-1 (TGM-1) enzyme. Clinical manifestation of IL is initiated with the occurrence of collodion membrane that sheds and is subsequenly replaced by thick scale, eclabium, ectropion, and palmoplantar hyperkeratosis. Gold standard of LI diagnosis is genetic analysis examination. Ichthyosis therapy includes scales removal, topical keratolytic, oral retinoid, and its complication management. Case: A boy of 3 years and 11 months old was referred from Paediatric Polyclinic to Dermatology and Venereology Polyclinic of Dr. Moewardi Hospital, Surakarta with an ailment of scales all over his body. The scales have appeared since birth. The patient was born encased by a membrane that subsequently shed in a few days, afterward the skin almost all over his body got dry, thicken, and scally li...
