Lamellar Ichthyosis
Nils Olof Sandberg MD1
1 Department of Pediatrics, Hospital of Aengelholm, Aengelholm, Sweden
Congenital ichthyoses represent a heterogeneous group of rare skin diseases. The disorders are heritable and have in common a varying degree of hyperkeratosis. During the years there has been much confusion regarding the classification of the ichthyoses. Today we have a classification based on clinical, genetic, cellular, and kinetic observations (Table). 1,2 Most patients fit into one of the four major types of ichthyoses. Beside those major types, congenital ichthyosis may be seen as part of several uncommon syndromes. The most often seen congenital ichthyosis is the lamellartype which is inherited as an autosomal recessive trait.3
Lamellar ichthyosis was first described by Seeligmann under the title of "epidermal desquamation of the newborn" in 1841.4 Lentz and Altman reviewed the literature in 1968 and found a total of 103 cases.5
CASE HISTORY
A.S., a 2,720-gm infant was born at 37 weeks of gestation. His parents and a brother are healthy. Pregnancy and labor were uncomplicated. Apgar scores at one and five minutes were 10.
At birth the infant was encased in a shiny collodion membrane. Because of the tight envelope his nose, ears, fingers, and toes were whitish, and the baby could hardly move. The lips and eyelids were everted. The diagnosis of collodion baby was made immediately (Figs 1 and 2).
