Epidermolysis bullosa pruriginosa

Common Name(s)

Epidermolysis bullosa pruriginosa

Dystrophic epidermolysis bullosa is an inherited skin fragility disorder associated with anchoring fibril abnormalities and sublamina densa blistering. EB pruriginosa is a rare distinct clinical subtype of dystrophic EB in which skin fragility, blistering, and scar formation are associated with intense pruritus, nodular prurigo-like lichenified lesions, nail dystrophy, and variable presence of albopapuloid lesions ({5:McGrath et al., 1994}; {1:Cambiaghi et al., 1997}). The onset of these clinical features may be evident in early childhood, but in some cases is delayed until the second or third decade of life. Autosomal dominant, autosomal recessive, and sporadic inheritance patterns have all been described in this disorder.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Epidermolysis bullosa pruriginosa" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Epidermolysis bullosa pruriginosa" returned 10 free, full-text research articles on human participants. First 3 results:

Treatment of epidermolysis bullosa pruriginosa using systemic and topical agents.
 

Author(s): Aaron R Mangold, Christine M Cole, David J DiCaudo, Mark R Pittelkow, Aleksandar Sekulic

Journal: J. Am. Acad. Dermatol.. 2014 Jun;70(6):e136-7.

 

Last Updated: 16 May 2014

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Epidermolysis bullosa pruriginosa: a rare presentation with asymptomatic lesions.
 

Author(s): Sangita Ghosh, Soumik Chaudhuri, Vijay Kumar Jain

Journal: Indian J Dermatol Venereol Leprol. ;79(2):235-7.

 

Epidermolysis bullosa pruriginosa (EBP) is a subtype of dominant dystrophic epidermolysis bullosa (DDEB) and is clinically characterized by pruritic lichenified plaques or prurigo-like lesions with violaceous linear scarring. Pruritus has always been described as one of the most striking ...

Last Updated: 27 Feb 2013

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An incompletely penetrant novel mutation in COL7A1 causes epidermolysis bullosa pruriginosa and dominant dystrophic epidermolysis bullosa phenotypes in an extended kindred.
 

Author(s): Catherine S Yang, Yin Lu, Anita Farhi, Carol Nelson-Williams, Michael Kashgarian, Earl J Glusac, Richard P Lifton, Richard J Antaya, Keith A Choate

Journal: Pediatr Dermatol. ;29(6):725-31.

 

Epidermolysis bullosa pruriginosa (EBP) is a rare subtype of dystrophic epidermolysis bullosa (DEB) characterized by intense pruritus, nodular or lichenoid lesions, and violaceous linear scarring, most prominently on the extensor extremities. Remarkably, identical mutations in COL7A1, ...

Last Updated: 30 Oct 2012

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Reviews from the PubMed Database

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The terms "Epidermolysis bullosa pruriginosa" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

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