Dystrophic epidermolysis bullosa pruriginosa: a new case series of a rare phenotype unveils skewed Th2 immunity

2021 ◽  
Author(s):  
Delphine Darbord ◽  
Geoffroy Hickman ◽  
Nathalie Pironon ◽  
Claire Barbieux ◽  
Mathilde Bonnet‐des‐Claustres ◽  
...  
Keyword(s):  
Epidermolysis Bullosa ◽  
Case Series ◽  
Dystrophic Epidermolysis Bullosa ◽  
Rare Phenotype ◽  
Th2 Immunity

scholarly journals 183 Dystrophic epidermolysis bullosa pruriginosa: a new case series of a rare phenotype unveils skewed Th2 immunity

2021 ◽  
Vol 141 (10) ◽  
pp. S179
Author(s):  
D. Darbord ◽  
G. Hickman ◽  
N. Pironon ◽  
C. Barbieux ◽  
M. Bonnet-des-Claustres ◽  
...  
Keyword(s):  
Epidermolysis Bullosa ◽  
Case Series ◽  
Dystrophic Epidermolysis Bullosa ◽  
Rare Phenotype ◽  
Th2 Immunity

scholarly journals Intrafamilial Diversity of Clinical Severity in Dominant Dystrophic Epidermolysis Bullosa: Case Series of Three Generations

2019 ◽  
Vol 13 (1) ◽  
pp. 3-7
Author(s):  
Inne Arline Diana ◽  
Srie Prihianti Gondokaryono ◽  
Reiva Farah Dwiyana ◽  
July Iriani Rahardja ◽  
Yuri Yogya ◽  
...  
Keyword(s):  
Epidermolysis Bullosa ◽  
Autosomal Recessive ◽  
Autosomal Dominant ◽  
Case Series ◽  
Clinical Severity ◽  
Dystrophic Epidermolysis Bullosa ◽  
Tissue Separation ◽  
Three Generations ◽  
Unknown Gene ◽  
Main Observation

Background: Epidermolysis bullosa, a group of inheritable blistering diseases with considerable clinical and genetic diversity, is divided into distinct subtypes depending on the level of tissue separation in the dermal–epidermal basement membrane zone. The dystrophic form of epidermolysis bullosa (DEB) is characterized by tense blisters and erosions which heals with extensive scarring. The fact that DEB can be inherited in either autosomal dominant (DDEB) or autosomal recessive (RDEB) pattern adds to its clinical diversity. The cause of marked clinical diversity in mild to severe DDEB is still unidentified. Main Observation: We report an intrafamilial diversity of clinical severity in dominant dystrophic epidermolysis bullosa (DDEB) cases within three generations. Conclusion: We emphasize the variety of clinical severity in DDEB cases within three generations which might be caused by unknown gene modifiers and environmental factors.

scholarly journals DYSTROPHIC EPIDERMOLYSIS BULLOSA - CASE SERIES OF CRYSTAL SKIN CHILDREN

2020 ◽  
pp. 1-2
Author(s):  
S. Madhulika ◽  
Sreekala. R ◽  
Uday Kiran Raja ◽  
A.Vijaya Mohan Rao
Keyword(s):  
Gene Therapy ◽  
Nursing Care ◽  
Epidermolysis Bullosa ◽  
Multidisciplinary Approach ◽  
Histopathological Examination ◽  
Case Series ◽  
Effective Management ◽  
Inherited Disorders ◽  
Dystrophic Epidermolysis Bullosa ◽  
Disorder Treatment

Epidermolysis bullosa (EB) refers to a group of inherited disorders that involve the formation of blisters following trivial trauma. Here we report a case series of Dystrophic Epidermolysis bullosa(DEB) at our hospital presented with various clinical features. History ,clinical spectrum and histopathological examination gives appropriate diagnosis for this disorder. Treatment remains challange. A multidisciplinary approach is needed for the effective management of EB. Good nursing care, and gene therapy could possibly significantly alleviate the suffering of the patients in the future.

Early teeth extraction in patients with generalized recessive dystrophic epidermolysis bullosa: A case series

2020 ◽  
Vol 40 (6) ◽  
pp. 561-565
Author(s):  
Sebastián Véliz ◽  
Hinrich Huber ◽  
María Joao Yubero ◽  
Ignacia Fuentes ◽  
Fatimah Alsayer ◽  
...  
Keyword(s):  
Epidermolysis Bullosa ◽  
Case Series ◽  
Dystrophic Epidermolysis Bullosa ◽  
Recessive Dystrophic Epidermolysis Bullosa ◽  
Teeth Extraction

Congenital dystrophic epidermolysis bullosa (DEB) in Sprague Dawley rats: a case series

2016 ◽  
Vol 27 (2) ◽  
pp. 122-e34 ◽  
Author(s):  
Kristin B. Eden ◽  
Ashley Peterson ◽  
Harold R. Payne ◽  
Wayne V. Corapi ◽  
Joanne Mansell ◽  
...  
Keyword(s):  
Epidermolysis Bullosa ◽  
Case Series ◽  
Sprague Dawley ◽  
Dystrophic Epidermolysis Bullosa ◽  
Sprague Dawley Rats
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