Dermatology Book

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Ichthyosis

Aka: Ichthyosis, Lamellar Ichthyosis, Congenital Ichthyosiform Erythroderma, Epidermolytic Hyperkeratosis, X-Linked Ichthyosis, Ichthyosis Vulgaris
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  1. See Also
    1. Xerotic Eczema
    2. Eczematous Dermatitis
    3. Dyshidrotic Eczema
  2. Causes
    1. Hereditary disease
      1. Ichthyosis Vulgaris
      2. X-Linked Ichthyosis
      3. Refsum's Syndrome
        1. Hereditary mental deficiency
        2. Spastic paralysis
    2. Congenital disease
      1. Lamellar Ichthyosis (Non-bullous)
      2. Epidermolytic Hyperkeratosis (Bullous)
    3. Sjogren-Larsson Syndrome
    4. Leprosy
    5. Hypothyroidism
    6. AIDS
  3. Pathophysiology
    1. Dysfunctional sweating and oil secretion
  4. Symptoms
    1. Skin dryness (worse in winter)
    2. Failed sweating
  5. Signs
    1. Skin changes
      1. Dry and brittle
      2. Cracked, thickened, and Scaling
    2. No sweating or oil secretion
  6. Management
    1. See Dry Skin Management
  7. Resources
    1. Icthyosis Information
      1. http://www.ichthyosis.com/
    2. First Foundation for Icthyosis
      1. http://www.scalyskin.org

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 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, Ichthyosis vulgaris, eritrodermia ictiosiforme congénita dominante (trastorno), eritrodermia ictiosiforme congénita dominante, ictiosis vulgar (trastorno), ictiosis vulgar dominante, ictiosis vulgaris, ictiosis vulgar, Ictiosis Vulgar
Swedish Fiskfjällssjuka
Japanese ｼﾞﾝｼﾞｮｳｾｲｷﾞｮﾘﾝｾﾝ, 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
French Ichtyose vulgaire, Ichtyose vulgaire autosomique dominante
German Ichthyosis vulgaris
Italian Ittiosi volgare
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Bullous Congenital Ichthyosiform Erythroderma (disorder) (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 Bullous Erythroderma Ichthyosiforme, Congenital ichthyosiform erythroderma, bullous, Epidermolytic Hyperkeratoses, Erythroderma Ichthyosiforme, Bullous, Hyperkeratoses, Epidermolytic, Hyperkeratosis, Epidermolytic, Bullous Congenital Ichthyosiform Erythroderma, Congenital Bullous Ichthyosiform Erythroderma, Ichthyosiform Erythroderma, Bullous Congenital, Hyperkeratosis, epidermolytic, 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, Epidermolytic Hyperkeratosis, Bullous Congenital Ichthyosiform Erythroderma (disorder), 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, 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, Hiperqueratosis Epidermolitica, Eritrodermia Ictiosiforme Bullosa Congenita
Japanese 表皮溶解性角化症, ｽｲﾎｳｾｲｷﾞ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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Congenital ichthyosis (C0020758)

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 C538579
ICD9 757.1
ICD10 Q80, Q80.9
SnomedCT 268283000, 205547001, 13059002, 157017000, 268355000, 205556009
English congenital ichthyosis, Congenital ichthyosis of skin, NOS, Fish skin, NOS, Congenital ichthyosis unspec., Congenital ichthyosis, unspecified, Ichthyosis congenita NOS, ICHTHYOSIS CONGENITA, LI, ICHTHYOSIS CONGENITA II, ICHTHYOSIS, LAMELLAR, 1, COLLODION FETUS, LAMELLAR ICHTHYOSIS, TYPE 1, LAMELLAR ICHTHYOSIS, DESQUAMATION OF NEWBORN, LI1, LAMELLAR EXFOLIATION OF NEWBORN, ICR2, ichthyosis congenita, Congenital ichthyosis, unspecified (disorder), Ichthyosis congenita NOS (disorder), Lamellar exfoliation of newborn, Lamellar ichthyosis, type 1, Desquamation of newborn, Collodion fetus, fish skin, 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, Ichthyosis, NOS, Ichthyosis congenital, ichthyosis
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, Ichthyosis congenita NOS, Ichthyosis congenita, enfermedad de la escama de pescado, ictiosis congénita de la piel (trastorno), ictiosis congénita de la piel, ictiosis congénita, SAI (trastorno), ictiosis congénita, SAI, ictiosis congénita, no especificada (trastorno), ictiosis congénita, no especificada, ictiosis congénita, ictiosis, piel de pescado
Japanese 先天性魚鱗癬症, 先天性魚鱗癬, ｾﾝﾃﾝｾｲｷﾞｮﾘﾝｾﾝ, ｾﾝﾃﾝｾｲｷﾞｮﾘﾝｾﾝｼｮｳ
Czech Ichtyosis congenita, Vrozená ichtyóza, kongenitální ichtyóza
Korean 선천 비늘증, 상세불명의 선천 비늘증
Hungarian Ichthyosis congenita, Veleszületett ichthyosis
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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