II. Causes
-
Hereditary Disease
- Ichthyosis Vulgaris
- X-Linked Ichthyosis
- Refsum's Syndrome
- Hereditary mental deficiency
- Spastic paralysis
- Congenital disease
- Lamellar Ichthyosis (Non-bullous)
- Epidermolytic Hyperkeratosis (Bullous)
- Sjogren-Larsson Syndrome
- Leprosy
- Hypothyroidism
- AIDS
III. Pathophysiology
- Dysfunctional sweating and oil secretion
IV. Symptoms
- Skin dryness (worse in winter)
- Failed sweating
V. Signs
- Skin changes
- Dry and brittle
- Cracked, thickened, and Scaling
- No sweating or oil secretion
VI. Management
VII. Resources
- Icthyosis Information
- First Foundation for Icthyosis
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Definition (CSP) | skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis that exist at, and usually before, birth regardless of their causation; most ichthyoses are genetically determined. |
Concepts | Congenital Abnormality (T019) , Disease or Syndrome (T047) |
MSH | D017490 |
ICD9 | 757.1 |
ICD10 | Q80 , Q80.9 |
SnomedCT | 205547001, 268355000, 157017000, 268283000, 205556009, 13059002 |
English | Congenital ichthyosis unspec., Congenital ichthyosis, unspecified, Ichthyosis congenita NOS, ichthyosis congenita, ichthyosis, fish skin, congenital ichthyosis (diagnosis), congenital ichthyosis, ichthyosis congenital, Congenita Is, Ichthyosis, Lamellar Ichthyosis, Type 1, Ichthyosis Congenita, Newborn Lamellar Exfoliations, Lamellar Exfoliation of Newborn, Ichthyosis, Lamellar, 1, Is, Ichthyosis Congenita, Newborn Lamellar Exfoliation, Ichthyosis Congenita I, Congenita, Ichthyosis, Ichthyosis Congenita Is, Congenita I, Ichthyosis, Congenitas, Ichthyosis, I, Ichthyosis Congenita, Ichthyosis Congenitas, Congenital ichthyosis, unspecified (disorder), Ichthyosis congenita NOS (disorder), Ichthyosis, Ichthyosis congenita, Fish scale disease, Congenital ichthyosis of skin (disorder), Congenital ichthyosis of skin, Fish skin, disease (or disorder); fish-skin, fish-skin, Congenital ichthyosis, Congenital ichthyosis of skin, NOS, Fish skin, NOS, Ichthyosis, NOS, Ichthyosis congenital |
Dutch | ichthyosis congenita, ichtyose, congenitaal, congenitale ichthyosis, aandoening; vissenhuid, vissenhuid; aandoening, Congenitale ichthyose, niet gespecificeerd, Congenitale ichthyose |
French | Ichtyose congénitale, Ichthyose congénitale |
German | kongenitale Ichthyose, Ichthyosis kongenital, Ichthyosis congenita, Ichthyosis congenita, nicht naeher bezeichnet |
Italian | Ittiosi congenita |
Portuguese | Ictiose congénita |
Spanish | Ictiosis congénita, ictiosis congénita, SAI, ictiosis congénita, no especificada, ictiosis congénita, SAI (trastorno), ictiosis congénita, no especificada (trastorno), enfermedad de la escama de pescado, ictiosis congénita de la piel (trastorno), ictiosis congénita de la piel, ictiosis congénita, ictiosis, piel de pescado |
Japanese | 先天性魚鱗癬症, 先天性魚鱗癬, センテンセイギョリンセン, センテンセイギョリンセンショウ |
Czech | Ichtyosis congenita, Vrozená ichtyóza, kongenitální ichtyóza |
Korean | 선천 비늘증, 상세불명의 선천 비늘증 |
Hungarian | Ichthyosis congenita, Veleszületett ichthyosis |
Ontology: Hyperkeratosis, Epidermolytic (C0079153)
Definition (NCI) | An autosomal dominant inherited skin disorder caused by mutations in the KRT1 and KRT10 genes. It is manifested at birth and is characterized by generalized erythema, skin blisters and skin fragility. |
Definition (MSH) | A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by ERYTHRODERMA and severe hyperkeratosis. It is manifested at birth by blisters followed by the appearance of thickened, horny, verruciform scales over the entire body, but accentuated in flexural areas. Mutations in the genes that encode KERATIN-1 and KERATIN-10 have been associated with this disorder. |
Concepts | Congenital Abnormality (T019) , Disease or Syndrome (T047) |
MSH | D017488 |
ICD10 | Q80.3 |
SnomedCT | 20512000, 239071005, 254167000 |
English | Epidermolytic Hyperkeratoses, Erythroderma Ichthyosiforme, Bullous, Hyperkeratoses, Epidermolytic, Bullous Congenital Ichthyosiform Erythroderma, Congenital Bullous Ichthyosiform Erythroderma, Ichthyosiform Erythroderma, Bullous Congenital, Bull ichthyosform erythroderma, Congenital Ichthyosiform Erythroderma, Bullous, BULLOUS ICHTHYOSIFORM ERYTHRODERMA, BULLOUS CONGENITAL ICHTHYOSIFORM ERYTHRODERMA, BCIE, BIE, EHK, ICHTHYOSIFORM ERYTHRODERMA BULLOUS CONGEN, CONGEN BULLOUS ICHTHYOSIFORM ERYTHRODERMA, BULLOUS CONGEN ICHTHYOSIFORM ERYTHRODERMA, BIE - Bull ichthy erythroderma, Bullous Ichthyosiform Erythroderma Congenital, Bullous Erythroderma Ichthyosiformes, Ichthyosiforme, Bullous Erythroderma, Erythroderma Ichthyosiformes, Bullous, Ichthyosiformes, Bullous Erythroderma, BULLOUS ERYTHRODERMA ICHTHYOSIFORMIS CONGENITA OF BROCQ, EPIDERMOLYTIC HYPERKERATOSIS, Hyperkeratosis, Epidermolytic [Disease/Finding], epidermolytic hyperkeratosis, EPIDERMOLYTIC ICHTHYOSIS, Bullous Ichthyosiform Erythroderma, Bullous Erythroderma Ichthyosiformis Congenita of Brocq, Erythrodermas, Bullous Ichthyosiform, Ichthyosiform Erythroderma, Bullous, Bullous Ichthyosiform Erythrodermas, Erythroderma, Bullous Ichthyosiform, Ichthyosiform Erythrodermas, Bullous, Epidermolytic Hyperkeratosis, congenital bullous ichthyosiform erythroderma (diagnosis), Hyperkeratosis, Epidermolytic, Bullous Erythroderma Ichthyosiforme, Epidermolytic Ichthyosis, Congenital bullous ichthyosiform erythroderma, Epidermolytic hyperkeratosis, Epidermolytic palmoplantar hyperkeratosis, Bullous ichthyosiform erythroderma, BIE - Bullous ichthyosiform erythroderma, Bullous ichthyosiform erythroderma (disorder), erythema; ichthyosiforme congenitum bullosum, ichthyosiforme congenitum bullosum; erythema |
Dutch | bulleuze ichthyosiforme erythroderma, epidermolytische hyperkeratosis, erytheem; ichthyosiforme congenitum bullosum, ichthyosiforme congenitum bullosum; erytheem, Congenitale bulleuze ichthyosiforme erytrodermie, Epidermolytische hyperkeratosis, Erytrodermie, bulleuze ichthyosiforme, Hyperkeratosis, epidermolytische, Ichthyosiforme bulleuze erytrodermie |
French | Erythrodermie ichthyosiforme bulleuse, Érythrodermie ichtyosiforme bulleuse, Hyperkératose épidermolytique, Érythrodermie ichtyosiforme congénitale bulleuse |
German | epidermolytische Hyperkeratose, bulloese ichthyotische Erythrodermie, Bulloese kongenitale ichthyosiforme Erythrodermie, Hyperkeratose, epidermolytische, Erythroderma ichthyosiforme, bullöses, Ichthyosiforme Erythrodermie, bullöse kongenitale |
Italian | Eritroderma ittiosiforme bolloso, Eritoderma ittiosiforme bolloso congenito, Eritoderma ittiosiforme bolloso, Ipercheratosi epidermolitica |
Portuguese | Hiperqueratose epidermolítica, Eritrodermite bolhosa ictiosiforme, Eritrodermia Ictiosiforme Bolhosa Congênita, Eritrodermia Ictiosiforme Bolhosa, Hiperceratose Epidermolítica |
Spanish | Eritrodermia ictiosiforme ampolloso, Hiperqueratosis epidermolítica, eritrodermia ictiosiforme bullosa (trastorno), eritrodermia ictiosiforme bullosa congénita, eritrodermia ictiosiforme bullosa, hiperqueratosis epidermolítica, Eritrodermia Ictiosiforme Bullosa Congénita, Eritrodermia Ictiosiforme Bullosa, Hiperqueratosis Epidermolítica |
Japanese | 表皮溶解性角化症, スイホウセイギョリンセンヨウコウヒショウ, ヒョウヒヨウカイセイカクカショウ, ヒョウヒヨウカイセイカッカショウ, 角化症-表皮剥離性, 水疱型先天性魚鱗癬様紅皮症, 水疱型魚鱗癬様紅皮症, 水疱性先天性魚鱗癬様紅皮症, 水疱性魚鱗癬様紅皮症, 表皮剥離性角化症, 表皮剥離性角質増殖症, 魚鱗癬様紅皮症-水疱型, 魚鱗癬様紅皮症-水疱型先天性, 魚鱗癬様紅皮症-水疱性, 魚鱗癬様紅皮症-水疱性先天性 |
Swedish | Hyperkeratos, epidermolytisk |
Czech | erythrodermia ichthyosiformis bullosa congenitalis, erythrodermia ichthyosiformis bullosa, hyperkeratóza epidermolytická, Bulózní ichthyosiformní erytrodermie, Epidermolytická hyperkeratóza |
Finnish | Epidermolyyttinen hyperkeratoosi |
Russian | IKHTIOZIFORMNAIA ERITRODERMIIA BULLEZNAIA VROZHDENNAIA, GIPERKERATOZ EPIDERMOLITICHESKII, ERITRODERMIIA IKHTIOZIFORMNAIA BULLEZNAIA, ГИПЕРКЕРАТОЗ ЭПИДЕРМОЛИТИЧЕСКИЙ, ИХТИОЗИФОРМНАЯ ЭРИТРОДЕРМИЯ БУЛЛЕЗНАЯ ВРОЖДЕННАЯ, ЭРИТРОДЕРМИЯ ИХТИОЗИФОРМНАЯ БУЛЛЕЗНАЯ |
Korean | 선천 수포성 비늘증성 홍색피부증 |
Polish | Erytrodermia pęcherzowa ichtiotyczna wrodzona, Erytrodermia ichtiotyczna pęcherzowa |
Hungarian | Epidermolytic hyperkeratosis, Bullosus ichthyosiform erythroderma |
Norwegian | Medfødt bulløs iktyosiform erytrodermi, Epidermolytisk hyperkeratose |
Ontology: Congenital Nonbullous Ichthyosiform Erythroderma (C0079154)
Definition (NCI) | A very rare, autosomal recessive inherited skin disorder present at birth. It is characterized by the presence of a transparent membrane encasing the newborn. This membrane sheds in about two weeks after birth to reveal generalized scaling and skin erythema. |
Definition (MSH) | A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate. |
Concepts | Congenital Abnormality (T019) |
MSH | D017490 |
ICD10 | Q80.2 |
SnomedCT | 205550003, 267372009, 268245001, 35970001, 12215009 |
English | Erythroderma Ichthyosiforme, Nonbullous, Ichthyosis, Lamellar, Nonbullous Erythroderma Ichthyosiforme, Nonbullous Congenital Ichthyosiform Erythroderma, Ichthyosiform Erythroderma, Nonbullous Congenital, CONGEN NONBULLOUS ICHTHYOSIFORM ERYTHRODERMA, NONBULLOUS CONGEN ICHTHYOSIFORM ERYTHRODERMA, ICHTHYOSIFORM ERYTHRODERMA NONBULLOUS CONGEN, Congenital ichthyosis, Ichthyosiforme, Nonbullous Erythroderma, Ichthyosiformes, Nonbullous Erythroderma, Nonbullous Erythroderma Ichthyosiformes, Congenital Ichthyosiform Erythroderma, Nonbullous, Nonbullous Congenital Lamellar Ichthyosis, Erythroderma Ichthyosiformes, Nonbullous, Congenital Nonbullous Ichthyosiform Erythroderma, Ichthyosis, Lamellar [Disease/Finding], congenital ichthyosis, lamellar ichthyosis, ichthyosis lamellar, Lamellar ichthyosis (diagnosis), Ichthyosis, congenital, Nonbullous congenital ichthyosiform erythroderma, Lamellar Ichthyosis, Lamellar Ichthyoses, Ichthyoses, Lamellar, Lamellar Ichthyose, Ichthyose, Lamellar, Collodion baby, Congenital non bullous ichthyosiform erythroderma, Lamellar desquamation of the newborn, Lamellar ichthyosis, Congenital non bullous ichthyosiform erythroderma (disorder), Lamellar ichthyosis (disorder), collodion baby, ichthyosis; lamellar, lamellar; ichthyosis |
Dutch | lamellaire ichthyosis, collodiumbaby, ichthyose; lamellair, lamellair; ichthyose, Erythroderma ichthyosiforme, niet-bulleus, Ichthyose, lamellaire, Ichthyosiforme erythroderma, non-bulleuze, congenitale, Lamellaire ichthyose |
French | Ichthyose lamellaire, Érythrodermie ichtyosiforme non bulleuse, Ichtyose lamellaire, Érythrodermie ichtyosiforme congénitale non bulleuse, Bébé collodion |
German | lamellaere Ichthyosis, Lamellaere Ichthyosis, Collodium-Baby, Erythrodermie, ichthyosiforme, nichtbullöse, Ichthyose, lamelläre, Ichthyosiforme Erythrodermie, nichtbullöse, kongenitale |
Portuguese | Ictiose lamelar, Bebé colódio, Eritrodermia Ictiosiforme não-Bolhosa Congênita, Eritrodermia Ictiosiforme não-Bolhosa, Ictiose Lamelar |
Spanish | Ictiosis lamelar, ictiosis lamelar (trastorno), ictiosis laminar (trastorno), ictiosis laminar, ictiosis congénita, feto de arlequín (trastorno), feto de arlequín, bebé colodiano, descamación laminillar del recién nacido, ictiosis lamelar, eritrodermia ictiosiforme no bullosa congénita, Descamación laminar del recién nacido, bebe colodión, descamación lamelar del neonato, eritrodermia ictiosiforme bullosa no congénita (trastorno), eritrodermia ictiosiforme bullosa no congénita, feto de arlequín (concepto no activo), Eritrodermia Ictiosiforme no Bullosa Congénita, Eritrodermia Ictiosiforme no Bullosa, Ictiosis Lamelar |
Swedish | Iktyos, lamellär |
Japanese | ソウジョウギョリンセン, 魚鱗癬様紅皮症-非水疱性先天性, 葉状魚鱗癬, 魚鱗癬様紅皮症-非水疱性, 非水疱性魚鱗癬様紅皮症, 層状魚鱗癬, 非水疱型先天性魚鱗癬様紅皮症, 非水疱性先天性魚鱗癬様紅皮症, 魚鱗癬-層状, 魚鱗癬-葉状, コロジオンベイビー, コロジオンベイビー |
Czech | ichtyóza lamelární, erythrodermia ichthyosiformis nonbullosa, erythrodermia ichthyosiformis nonbullosa congenitalis, Lamelární ichtyóza, Syndrom koloidního novorozence |
Finnish | Lamellaarinen kalansuomutauti |
Russian | ERITRODERMIIA IKHTIOZIFORMNAIA NEBULLEZNAIA, LISHAI PLASTINCHATYI, IKHTIOZ CHESHUICHATYI, IKHTIOZIFORMNAIA ERITRODERMIIA NEBULLEZNAIA VROZHDENNAIA, ИХТИОЗИФОРМНАЯ ЭРИТРОДЕРМИЯ НЕБУЛЛЕЗНАЯ ВРОЖДЕННАЯ, ИХТИОЗ ЧЕШУЙЧАТЫЙ, ЛИШАЙ ПЛАСТИНЧАТЫЙ, ЭРИТРОДЕРМИЯ ИХТИОЗИФОРМНАЯ НЕБУЛЛЕЗНАЯ |
Italian | Eritrodermia ittiosiforme non bollosa, Eritrodermia congenita ittiosiforme non bollosa, Ittiosi lamellare neonatale, Ittiosi lamellare |
Korean | 층판성 비늘증 |
Polish | Erytrodermia ichtiotyczna bezpęcherzowa, Rybia łuska blaszkowata, Erytrodermia ichtiotyczna bezpęcherzowa wrodzona |
Hungarian | Lamelláris ichthyosis, ((collodion)) csecsemő |
Norwegian | Lammelær iktyose, Medfødt ikke-bulløs iktyosiform erytrodermi |
Ontology: Ichthyosis Vulgaris (C0079584)
Definition (NCI) | The most common form of ichthyosis. It is an autosomal dominant inherited or acquired disorder characterized by scaling and desquamation of the skin. |
Definition (MSH) | Most common form of ICHTHYOSIS characterized by prominent scaling especially on the exterior surfaces of the extremities. It is inherited as an autosomal dominant trait. |
Concepts | Congenital Abnormality (T019) , Disease or Syndrome (T047) |
MSH | D016112 |
ICD10 | Q80.0 |
SnomedCT | 205551004, 254157005, 20512000 |
English | Dominant Ichthyosis Vulgaris, Ichthyosis Vulgaris, Dominant, ICHTHYOSIS SIMPLEX, ICHTHYOSIS VULGARIS, ichthyosis vulgaris (diagnosis), ichthyosis vulgaris, Ichthyosis Vulgaris [Disease/Finding], Ichthyosis Simplex, Ichthyosis Simplices, Ichthyosis vulgaris, Dominant congenital ichthyosiform erythroderma, Dominant ichthyosis vulgaris, Dominant congenital ichthyosiform erythroderma (disorder), Ichthyosis vulgaris (disorder), ichthyosis; simplex, ichthyosis; vulgaris, simplex; ichthyosis, vulgaris; ichthyosis, Ichthyosis Vulgaris |
Dutch | ichthyosis vulgaris, ichthyose; simplex, ichthyose; vulgaris, simplex; ichthyose, vulgaris; ichthyose, Ichthyosis vulgaris |
Portuguese | Ictiose vulgar, Ictiose Vulgar |
Spanish | Ictiosis vulgar, eritrodermia ictiosiforme congénita dominante (trastorno), eritrodermia ictiosiforme congénita dominante, ictiosis vulgar (trastorno), ictiosis vulgar dominante, ictiosis vulgaris, ictiosis vulgar, Ictiosis Vulgar |
Swedish | Fiskfjällssjuka |
Japanese | ジンジョウセイギョリンセン, 魚鱗癬-尋常性, 優性遺伝性尋常性魚鱗癬, 尋常性魚鱗癬, 尋常魚鱗癬 |
Czech | ichthyosis vulgaris, Ichtyosis vulgaris |
Finnish | Yleinen kalansuomutauti |
Russian | IKHTIOZ OBYKNOVENNYI, ИХТИОЗ ОБЫКНОВЕННЫЙ |
Korean | 보통 비늘증 |
Polish | Rybia łuska prosta, Rybia łuska zwykła |
Hungarian | Ichthyosis közönséges |
Norwegian | Dominant ichthyosis vulgaris, Ichthyosis simplex, Ichthyosis vulgaris |
French | Ichtyose simple, Ichtyose vulgaire, Ichtyose vulgaire autosomique dominante |
German | Ichthyosis vulgaris |
Italian | Ittiosi volgare |
Ontology: Ichthyosis, X-Linked (C0079588)
Definition (CHV) | sex linked inherited disease characterized by excessive scaling |
Definition (CHV) | sex linked inherited disease characterized by excessive scaling |
Definition (CHV) | sex linked inherited disease characterized by excessive scaling |
Definition (CHV) | sex linked inherited disease characterized by excessive scaling |
Definition (CHV) | sex linked inherited disease characterized by excessive scaling |
Definition (CHV) | sex linked inherited disease characterized by excessive scaling |
Definition (NCI) | The second most common form of ichthyosis. It is an X-linked inherited disorder with mild skin manifestations. The skin changes appear at birth and include keratinization and scaling. |
Definition (MSH) | Chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the X-chromosome and transmitted to the male offspring. It is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency. |
Concepts | Disease or Syndrome (T047) |
MSH | D016114 |
ICD10 | Q80.1 |
SnomedCT | 205552006, 254160003, 72523005, 402771003 |
English | Ichthyoses, Sex-Linked, Ichthyoses, X-Linked, Ichthyosis, Sex Linked, Ichthyosis, Sex-Linked, Ichthyosis, X Linked, Ichthyosis, X-Linked, ICHTHYOSIS, X-LINKED, XRI - X-linked rec ichthyosis, XRI - X-linked recessive ichthyosis, XLI, Ichthyosis, X-linked, X-Linked Ichthyosis, Ichthyosis, X-Linked [Disease/Finding], x-linked ichthyosis, x-linked recessive ichthyosis, sts, ssdd, x linked ichthyosis, X-linked ichthyosis (STS, SSDD), ichthyosis x-linked, X-linked ichthyosis (diagnosis), ichthyosis x-linked with steryl-sulfatase deficiency, X-linked ichthyosis with steryl-sulfatase deficiency (diagnosis), Sex-linked ichthyosis, X-linked ichthyosis, X-linked recessive ichthyosis, X-linked ichthyosis with steryl-sulfatase deficiency (disorder), X-linked ichthyosis with steryl-sulfatase deficiency, X-linked ichthyosis with steryl-sulphatase deficiency, X-linked recessive ichthyosis (disorder), X-linked; ichthyosis, ichthyosis; X-linked, X-linked recessive ichthyosis [Ambiguous] |
Dutch | X-gebonden ichthyosis, X-gebonden; ichthyose, ichthyose; X-gebonden, Ichthyose, X-gebonden, X-gebonden ichthyose |
French | Ichthyose liée à l'X, Ichtyose liée à l'X, Ichtyose liée au chromosome X, Ichtyose liée au sexe |
German | X-chromosal-rezessive Ichthyose, X-chromosomal-rezessive Ichthyosis, Ichthyose, X-chromosomal-gebundene, Ichthyose, geschlechtsgebundene |
Portuguese | Ictiose ligada ao cromossoma X, Doenças da Deficiência de Esteroide-Sulfatase, Ictiose Ligada ao Cromossomo X, Ictiose Ligada ao Sexo |
Spanish | Ictiosis ligada al cromosoma X, Enfermedad de la Deficiencia de Esteroide-Sulfatasa, ictiosis ligada al sexo (trastorno), ictiosis ligada al sexo, ictiosis recesiva relacionada con el cromosoma X (trastorno), ictiosis recesiva relacionada con el cromosoma X, Ictiosis Ligada al Cromosoma X, Ictiosis Ligada al Sexo |
Japanese | X−連鎖型魚鱗癬, Xレンサガタギョリンセン, 魚鱗癬-伴性遺伝性, X染色体関連魚鱗症, X染色体性魚鱗癬, X染色体連関魚鱗癬, X染色体関連魚鱗癬, 伴性遺伝性魚鱗癬, 伴性魚鱗癬, 魚鱗症-X染色体連関, 魚鱗癬-X染色体関連, 魚鱗症-X染色体関連, 魚鱗癬-X染色体性 |
Swedish | Iktyos, X-bunden |
Czech | ichtyóza vázaná na chromozóm X, X-vázaná ichtyóza |
Finnish | X-kromosomissa periytyvä kalansuomutauti |
Russian | IKHTIOZ X-SVIAZANNYI, IKHTIOZ, STSEPLENNYI S POLOM, ИХТИОЗ X-СВЯЗАННЫЙ, ИХТИОЗ, СЦЕПЛЕННЫЙ С ПОЛОМ |
Italian | Ittiosi legata al sesso, Ittiosi legata al cromosoma X |
Korean | 반성 비늘증 |
Polish | Rybia łuska sprzężona z płcią, Rybia łuska sprzężona z chromosomem X |
Hungarian | X-linked ichthyosis |
Norwegian | X-bundet iktyose, Kjønnsbundet iktyose |