Ichthyosis prematurity syndrome (IPS) is a rare autosomal recessive congenital ichthyosis characterized by the clinical triad of premature birth, scaly erythroderma, and neonatal asphyxia in combination with pathognomonic ultrastructural findings.1
Clinical features. A, Erythroderma with loose ichthyosiform desquamation 9 days after birth; the skin appears waxy and thickened, and palms show hyperlinearity. B, Marked improvement of the skin condition at age 15 weeks; only mild dryness and a faint erythema on the abdomen are still noticeable.
Microscopic images. Bars indicate 50 μm. A, Methylene blue–stained semithin section of a skin biopsy specimen from the buttocks, showing an orthohyperkeratotic corneal layer with lentiform swollen lamellae and a 2- to 3-layer stratum granulosum containing strikingly lucid globular cells. B-D, Electron micrographs presenting deposits of lipid membranes within the lamellae of the corneal layer; all bars indicate 1 μm. Cells of the granular layer appear remarkably translucent in perinuclear regions. C, In this higher magnification, the membranous aggregates, pathognomonic for ichthyosis prematurity syndrome, sometimes exhibit a trilamellar structure (arrows). D, Irregular membranous and vesicular material is seen in the granular layer.
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