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IchthyosisAka: 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

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.
ConceptsCongenital Abnormality (T019) , Disease or Syndrome (T047)
ICD9757.1, 757.1
EnglishCOLLODION FETUS, Congenital ichthyosis, Congenital ichthyosis of skin, DESQUAMATION OF NEWBORN, Fish scale disease, Fish skin, Ichthyosis, Ichthyosis congenita, ICHTHYOSIS CONGENITA II, ICR2, LAMELLAR EXFOLIATION OF NEWBORN, LAMELLAR ICHTHYOSIS, LI, LI1
Spanishenfermedad de la escama de pescado, ictiosis, ictiosis congenita, ictiosis congenita de la piel, piel de pescado
Parent ConceptsHereditary Diseases (C0019247), Skin Abnormalities (C0037268), Congenital ichthyosis (C0020758), Hereditary disorder of the integument (C1285303), Ambiguous concept (C1274012)
SourcesCSP, ICD9CM, MTH, MTHICD9, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Bullous Congenital Ichthyosiform Erythroderma (C0079153)

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.
ConceptsCongenital Abnormality (T019) , Disease or Syndrome (T047)
MSHD017488
EnglishBCIE, BIE, BIE - Bullous ichthyosiform erythroderma, BULLOUS CONGEN ICHTHYOSIFORM ERYTHRODERMA, Bullous Congenital Ichthyosiform Erythroderma, Bullous Erythroderma Ichthyosiforme, BULLOUS ERYTHRODERMA ICHTHYOSIFORMIS CONGENITA OF BROCQ, Bullous ichthyosiform erythroderma, CONGEN BULLOUS ICHTHYOSIFORM ERYTHRODERMA, Congenital Bullous Ichthyosiform Erythroderma, EHK, Epidermolytic Hyperkeratoses, Epidermolytic hyperkeratosis, Epidermolytic palmoplantar hyperkeratosis, ICHTHYOSIFORM ERYTHRODERMA BULLOUS CONGEN
Spanisheritrodermia ictiosiforme bullosa, eritrodermia ictiosiforme bullosa congenita, hiperqueratosis epidermolitica
Parent ConceptsIchthyosiform Erythroderma, Congenital (C0079583), Hyperkeratosis (C0870082), Bullous ichthyosis (C0432305)
SourcesMSH, MTH, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Ichthyosis Vulgaris (C0079584)

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.
ConceptsCongenital Abnormality (T019) , Disease or Syndrome (T047)
MSHD016112
EnglishDominant congenital ichthyosiform erythroderma, Dominant ichthyosis vulgaris, ICHTHYOSIS SIMPLEX, Ichthyosis Vulgaris
Spanisheritrodermia ictiosiforme congenita dominante, ictiosis vulgar, ictiosis vulgar dominante, ictiosis vulgaris
Parent ConceptsIchthyoses (C0020757), Skin Diseases, Genetic (C0037277), Congenital ichthyosis (C0020758), Duplicate concept (C1274013)
SourcesMSH, MTH, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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