Wang WH, Zhang L, Li LF, Sun TT. Ichthyosis hystrix Lambert type (IHL) and Curth-Macklin type (IHCM) are very rare conditions characterised by striking hyperkeratotic lesions. The patches of scaly, thickened skin range from spotty to severe, and may appear on almost any part of the body. The term ichthyosis hysterix is used to describe several rare skin disorders in the ichthyosis family of skin disorders characterized by massive hyperkeratosis with an appearance like spiny scales. Ichthyosis Hystrix (Lambert Type)a.k.a ichthyosis hystrix gravior, epidermolytic hyperkeratosis (EHK); bullous congenital ichthyosiform erythroderma. Inheritance is either autosomal dominant or sporadic. [1] This term is also used to refer to a type of epidermal nevi with extensive bilateral distribution. The first documented cases of familial IH were of Lambert type. Ichthyosis is a family of genetic skin disorders characterized by dry, thickened, scaly skin. Ichthyosis hystrix is a rare genodermatosis. Ichthyosis hystrix Lambert type (IHL) and Curth-Macklin type (IHCM) are very rare conditions characterised by striking hyperkeratotic lesions. Preferred Label: Ichthyosis hystrix gravior; Type: Other, mainly phenotypes with suspected mendelian basis; Alternative titles and symbols: Ichthyosis, lambert type; Porcupine man; Description: Anton-Lamprecht (1978) stated that 4 genetic disorders of keratinization are known to have a structural defect of tonofibrils. It is characterized by scaling skin (ichthyosis) ranging from mild to severe. Ichthyosis hystrix is a type of congenital ichthyosis associated with many rare conditions and presenting as generalized and nevoid forms. ichthyosis hystrix gravior (redirected from ichthyosis hystrix Lambert type ) An autosomal dominant condition (OMIM:146600) characterised by spiny scales covering the entire body (except the face, genitals, palms and soles), the only known cases of which were in Edward Lambert—“The Porcupine Man”, who was exhibited at the Royal Society in London in 1731—and in three generations of his … - ICHTHYOSIS HYSTRIX GRAVIOR - CURTH-MACKLIN TYPE, a disorder characterized by hystrix-like changes and keratoses of the palms and soles. It was first described in the Lambert family in Britain at the beginning of the 18th century. Ichthyosis hystrix, Curth-Macklin type is a rare inherited skin disorder. Ichthyosis hystrix: Introduction. Ultrastructural features of ichthyosis hystrix strongly resembling Lambert type. The first scientific, documented case was Edward Lambert, who was introduced to the Royal Society in London back in 1731. Indian Dermatol Online J 2013; 4:47. Ichthyosis hystrix (IH) is characterized by spiny hyperkeratotic scale, and includes Brocq type, Lambert type, Curth-Macklin type, Rheydt type and Bäfverstedt type. Lambert type Prevalence: - Inheritance: - Age of onset: - ICD-10: Q80.0 OMIM: 146600 UMLS: C0432311 MeSH: C536087 GARD: 9497 MedDRA: - Summary This disease has been moved to Ichthyosis hystrix of Curth-Macklin The documents contained in this web site are presented [orpha.net] The findings were presented at the "Südwestdeutschen Dermatologen-Kongress in Heidelberg 1976". Ichthyosis - NHS. Nayak S, Acharjya B, Mohanty P. Ichthyosis hystrix. Synonyymit: ichtyosis hystrix -käyttäytyminen, epidermolyyttinen hyperkeratoosi (EHK) tai bullous-synnynnäinen ichtyosiform erythroderma, Lambert-tyypin ichtyosis Nimi ichtyosis hystrix tulee kreikkalaisista sanoista icthyosis tarkoittaa kaloja ja kaloja hystrix se tarkoittaa kuin porcupine. Wen-Hui Wang 1, Long Zhang 2, Lin-Feng Li 1,3 & Synonyms: Ichthyosis congenita, Lamellar exfoliation of newborn, Desquamation of newborn, Collodion fetus, Lamellar ichthyosis, type … [1] [2]:771 Also known as ichthyosis hystrix gravior or porcupine man. About Ichthyosis Hystrix (Lambert Type) More: Evidence Summaries. ... Ichthyosis is a cutaneous disorder characterized by thick, dry, rough, and scaling skin, and autosomal recessive congenital ichthyosis (ARCI) is a nonsyndromic form of ichthyosis [1]. This disease is characterised by spiny scales which cover the entire body except the face, genitals, palms and soles. However, the ultrastructural features of IH Lambert type have not been reported. 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