Heterogeneity in autosomal recessive ichthyosis: clinical and biochemical differentiation of lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma

ML Williams, PM Elias - Archives of dermatology, 1985 - jamanetwork.com
• Nonbullous congenital ichthyosiform erythroderma (CIE) and classic lamellar ichthyosis
(LI) can be distinguished by clinical, histopathologic, and biochemical findings reported
herein. Whereas all patients with CIE (n= 12) had fine white scales, they were
heterogeneous with respect to degree of erythroderma, ectropion, and prognosis. In
contrast, all patients with LI (n= 6) had large, dark, platelike scales, severe ectropion that
showed no improvement with age, and minimal erythroderma. Although the stratum …