Lamellar Ichthyosis

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Abstract
Biopsy specimens from patients with lamellar ichthyosis were studied by histologic, enzyme histochemical, and electron microscopic methods. Routine sections were not diagnostic, showing hyperkeratosis, normal or thickened granular zone, and acanthosis and papillomatosis. Histochemical findings were intense acid phosphatase staining in the transitional zone and stratum corneum, and increased oxidative enzymes in epidermis. Electron microscopy showed increased numbers of keratinosomes in the upper stratum spinosum. Keratinization was abnormal, low density filaments being scant and ill-defined, and intercellular cement substance in the stratum corneum being increased. Mitochondria and nucleated cells persisted high in the granular layer. The enzyme histochemical staining and ultrastructure were consistent with previous reports, indicating not only an increased rate of epidermopoiesis in lamellar ichthyosis but also an increased adherence of the horny cells.