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Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma) Summary

146 relevant articles (1 outcomes, 6 trials/studies) found for this Disease

Description: 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.

Also Known As: Bullous Congenital Ichthyosiform Erythroderma; Congenital Bullous Ichthyosiform Erythroderma; Hyperkeratosis, Epidermolytic; Congenital Ichthyosiform Erythroderma, Bullous; Bullous Erythroderma Ichthyosiforme Show All >>

Key Drugs and Agents for Epidermolytic Hyperkeratosis

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. calcipotriene (calcipotriol) : 1 outcome in 1 result : FDA 4
  2. Keratins (Keratin) : 3 studies in 62 results : IBA
  3. Keratin-1 : 2 studies in 33 results : IBA
  4. Keratin-10 : 2 studies in 31 results : IBA
  5. Keratin-5 (Keratin 5) : 1 study in 1 result : IBA
  6. Arginine (L-Arginine) : 5 results : FDA 26
  7. Proline (L-Proline) : 4 results : FDA 25
  8. Etretinate : 4 results : FDA 1
  9. Collodion (Nitrocellulose) : 4 results : IBA
  10. Codon (Codons) : 4 results : IBA
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Diseases Related to Epidermolytic Hyperkeratosis

  1. Epidermolysis Bullosa Simplex (Epidermolysis Bullosa Herpetiformis Dowling Meara)
  2. Bloom Syndrome (Syndrome, Bloom)
  3. Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma)
  4. Ichthyosis (Xeroderma)
  5. Skin Diseases
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Key Therapies for Epidermolytic Hyperkeratosis

Efficacy Chart >>
  1. Hip Replacement Arthroplasty (Total Hip Replacement) : 1 result
  2. General Anesthesia : 1 result

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