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Junctional Epidermolysis Bullosa (Epidermolysis Bullosa Letalis)

Form of epidermolysis bullosa having onset at birth or during the neonatal period and transmitted through autosomal recessive inheritance. It is characterized by generalized blister formation, extensive denudation, and separation and cleavage of the basal cell plasma membranes from the basement membrane.
Also Known As:
Epidermolysis Bullosa Letalis; Epidermolysis Bullosa, Junctional; Epidermolysis Bullosa Junctionalis, Disentis Type; Epidermolysis Bullosa Junctionalis, Herlitz Type; Epidermolysis Bullosa Junctionalis, Progressive; Epidermolysis Bullosa Junctionalis, Severe Nonlethal; Epidermolysis Bullosa Progressiva; Epidermolysis Bullosa, Generalized Atrophic Benign; Epidermolysis Bullosa, Junctional, Herlitz Type; Epidermolysis Bullosa, Junctional, Herlitz-Pearson Type; Herlitz's Disease; Herlitz-Pearson Type Epidermolysis Bullosa; Herlitz-Pearson-Type Epidermolysis Bullosa; Bullosa Letali, Epidermolysis; Bullosa Letalis, Epidermolysis; Bullosa, Herlitz-Pearson-Type Epidermolysis; Disease, Herlitz; Disease, Herlitz's; Epidermolysis Bullosa Letali; Epidermolysis Bullosa, Herlitz-Pearson-Type; Herlitz Pearson Type Epidermolysis Bullosa; Herlitzs Disease; Letali, Epidermolysis Bullosa; Letalis, Epidermolysis Bullosa; Herlitz Disease; Lethal Junctional Epidermolysis Bullosa
Networked: 260 relevant articles (4 outcomes, 22 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Blister (Bulla)
2. Junctional Epidermolysis Bullosa (Epidermolysis Bullosa Letalis)
3. Skin Diseases (Skin Disease)
4. Epidermolysis Bullosa Dystrophica (Dystrophic Epidermolysis Bullosa)
5. Epidermolysis Bullosa

Experts

1. Has, Cristina: 12 articles (01/2022 - 12/2005)
2. Bruckner-Tuderman, Leena: 9 articles (11/2016 - 01/2002)
3. Schneider, Holm: 8 articles (01/2019 - 10/2002)
4. Zambruno, G: 8 articles (01/2018 - 11/2000)
5. Jonkman, M F: 8 articles (08/2016 - 05/2001)
6. Uitto, Jouni: 7 articles (10/2021 - 01/2002)
7. Meneguzzi, Guerrino: 6 articles (01/2006 - 09/2002)
8. Zambruno, Giovanna: 5 articles (11/2021 - 09/2003)
9. De Luca, Michele: 5 articles (01/2019 - 05/2006)
10. Pas, H H: 5 articles (08/2016 - 06/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Junctional Epidermolysis Bullosa:
1. KalininIBA
2. Monoclonal AntibodiesIBA
3. Hematoxylin (Haematoxylon)IBA
4. Eosine Yellowish-(YS) (Eosin)IBA
5. Phenytoin (Dilantin)FDA LinkGeneric
6. dupilumabIBA
7. Proteins (Proteins, Gene)FDA Link
8. NucleotidesIBA
9. Laminin (Merosin)IBA
10. Nonsense Codon (Nonsense Mutation)IBA

Therapies and Procedures

1. Therapeutics
2. Transplantation
3. Palliative Care (Palliative Therapy)
4. Cell- and Tissue-Based Therapy (Cell Therapy)
5. Pain Management