Congenital reticular ichthyosiform erythroderma--ichthyosis variegata: a case report and review of the literature.

We present a 32-year-old white patient with congenital reticular ichthyosiform erythroderma, also known as ichthyosis variegata. The patient had typical clinical features with areas of normal appearing skin surrounded by erythematous hyperkeratotic patches. The smaller "confetti-like" patches were mostly present on the trunk, while the larger areas forming a reticular pattern predominated on the extremities. Ultrastructural findings demonstrated perinuclear vacuolization, binuclear cells and filamentous deposits compatible with congenital reticular ichthyosiform erythroderma. Besides presenting the case, we review the literature on this rare disorder of keratinization.