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Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma)

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; Bullous Erythroderma Ichthyosiforme; Bullous Erythroderma Ichthyosiformes; Epidermolytic Hyperkeratoses; Erythroderma Ichthyosiformes, Bullous; Hyperkeratoses, Epidermolytic; Ichthyosiforme, Bullous Erythroderma; Ichthyosiformes, Bullous Erythroderma; Erythroderma Ichthyosiforme, Bullous; Ichthyosiform Erythroderma, Bullous Congenital
Networked: 173 relevant articles (2 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1. Akiyama, Masashi: 5 articles (05/2011 - 02/2002)
2. Shimizu, Hiroshi: 5 articles (05/2011 - 02/2002)
3. Sakai, Kaori: 3 articles (05/2011 - 06/2007)
4. Arin, Meral J: 3 articles (11/2009 - 01/2004)
5. Shwayder, Tor: 3 articles (11/2009 - 01/2004)
6. Ishida-Yamamoto, A: 3 articles (01/2002 - 08/2000)
7. Iizuka, H: 3 articles (01/2002 - 08/2000)
8. Takahashi, H: 3 articles (01/2002 - 08/2000)
9. Krieg, Thomas: 2 articles (11/2009 - 04/2006)
10. Hausser, Ingrid: 2 articles (11/2009 - 04/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Epidermolytic Hyperkeratosis:
1. Isotretinoin (Accutane)FDA LinkGeneric
2. calcipotriene (calcipotriol)FDA LinkGeneric
3. EmollientsIBA
4. Keratins (Keratin)IBA
5. Keratin-1IBA
6. Keratin-10IBA
7. Keratin-5 (Keratin 5)IBA
8. Bullous erythroderma ichthyosiformis congenita of BrocqIBA
9. Collodion (Nitrocellulose)IBA
10. Arginine (L-Arginine)FDA Link

Therapies and Procedures

1. General Anesthesia
2. Hip Replacement Arthroplasty (Total Hip Replacement)