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Article
March 1995

Absent Transglutaminase TGK Expression in Two of Three Patients With Lamellar Ichthyosis

Author Affiliations

Department of Dermatology University Hospital Leiden Rijnsburgerweg 10 2333 AA Leiden, the Netherlands

Toyama, Japan

Arch Dermatol. 1995;131(3):363-364. doi:10.1001/archderm.1995.01690150129034
Abstract

Lamellar ichthyosis is a disorder of cornification characterized by a congenital generalized nonbullous ichthyosis with a variable erythroderma and is probably composed of a heterogeneous group of various types. Classification is difficult because clear clinical, biochemical, or histopathologic parameters are not yet available.1

Recent data suggest that abnormalities of cornified envelope formation can occur in some types of lamellar ichthyosis. In an electron microscopic study, Niemi et al2 found a thin or absent cornified envelope in patients with recessive ichthyosis congenita type II. Van Hooijdonk et al3 showed an increase in membrane-bound transglutaminase TGK (TGK) in the scales of five patients with nonerythrodermic autosomal recessive lamellar ichthyosis. The TGK is an enzyme that is involved in the cornified envelope formation by catalyzing the cross-linking of precursor proteins such as involucrin. Hohl et al4 reported a reduction or absence of membrane-bound TGK in three patients by means of immunohistochemical

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