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Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma) Summary

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; Bullous Ichthyosiform Erythroderma Congenital; Congenital Ichthyosiform Erythroderma, Bullous Show All >>

Networked: 173 relevant articles (2 outcomes, 6 trials/studies) for this Disease

Key Drugs and Agents for Epidermolytic Hyperkeratosis

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Isotretinoin (Accutane) : 1 outcome in 3 results : FDA 6 Generic
  2. calcipotriene (calcipotriol) : 1 outcome in 2 results : FDA 9 Generic
  3. Emollients : 1 outcome in 1 result : IBA
  4. Keratins (Keratin) : 3 studies in 65 results : IBA
  5. Keratin-1 : 2 studies in 36 results : IBA
  6. Keratin-10 : 2 studies in 34 results : IBA
  7. Keratin-5 (Keratin 5) : 1 study in 1 result : IBA
  8. Bullous erythroderma ichthyosiformis congenita of Brocq : 6 results : IBA
  9. Collodion (Nitrocellulose) : 5 results : IBA
  10. Arginine (L-Arginine) : 5 results : FDA 19
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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. General Anesthesia : 2 results
  2. Hip Replacement Arthroplasty (Total Hip Replacement) : 1 result

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