The Ichthyoses

Prof. Dr. med. Heiko Traupe ist Oberarzt an der Klinik und Poliklinik für Hautkrankheiten der Universität Münster.

1 Introduction.- 1.1 Definition of the Term "Ichthyosis".- 1.2 History of the Ichthyoses.- 1.3 New Classifieation and Tables for Differential Diagnosis of the Ichthyoses.- 1.4 Epidermal Lipids and the Bioehemistry of Keratinization.- 2 Isolated Vulgar Ichthyoses.- 2.1 Autosomal Dominant Iehthyosis Vulgaris.- 2.2 X-Linked Reeessive Iehthyosis.- 3 Associated Ichthyoses of the Vulgaris Type.- 3.1 Refsum's Syndrome (Heredopathia Ataetiea Polyneuritiformis).- 3.2 Assoeiated Steroid-Sulfatase Deficieney.- 3.3 Iehthyosis and Hypogonadism: Reflections on the so-ealled Rud's Syndrome.- 3.4 Multiple Sulfatase Deficieney.- 4 Isolated Congenital Ichthyoses.- 4.1 Harlequin Fetus.- 4.2 The Lamellar Ichthyoses.- 4.3 Alopecia Ichthyotica: A Characteristic Feature of many Types of Congenital Ichthyosis.- 4.4 The Epidermolytic (Aeanthokeratolytic) Ichthyoses.- 5 Associated Congenital Ichthyoses.- 5.1 The Sjögren-Larsson Syndrome.- 5.2 Ichthyosis and Triehothiodystrophy: the Tay and PIBI(D)S Syndromes.- 5.3 The Comèl-Netherton Syndrome.- 5.4 X-Linked Dominant Ichthyosis.- 6 Recently Recognized Ichthyoses.- 6.1 Dorfman's Syndrome: Neutral Lipid Storage Disease with Ichthyotic Erythroderma.- 6.2 Hystrix-like Ichthyosis with Deafness: the HID Syndrome.- 6.3 Not an Ichthyosis at All: the Keratitis, Ichthyosis-like Hyperkeratosis, and Deafness (KID) Syndrome.- 6.4 The Ichthyosis Follicularis, Atrichia, and Photophobia (IFAP) Syndrome.- 6.5 Peeling-Skin Syndrome: Clinical and Morphological Evidence for Two Types.- 6.6 Autosomal Dominant Congenital Ichthyosis and Keratoderma Hereditaria Mutilans of Vohwinkel.- 6.7 Congenital Migratory Ichthyosis with Neurologic and Ophthalmologic Abnormalities.- 6.8 Ichthyoses of Uncertain Status.- 7 Therapy.- 7.1 Topical Therapy.- 7.2 SystemicTherapy.- 8 Subject Index.