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.

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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 15 free, full-text research articles on human participants. First 3 results:

Epidermolysis Bullosa Pruriginosa Excoriée: A Deceptive Pruritic Variant in Two Female Patients.
 

Author(s): Katarzyna B Gostyńska, Marcel F Jonkman

Journal: Acta Derm. Venereol.. 2016 Jan;96(1):140-1.

 

Last Updated: 20 Jan 2016

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Epidermolysis bullosa pruriginosa: a case with a novel mutation and co-existent lichen amyloidosus.
 

Author(s): Qiping Chen, Joyce Siong-See Lee, Hong Liang Tey

Journal: Indian J Dermatol Venereol Leprol. ;81(1):40-2.

 

Epidermolysis bullosa pruriginosa is a rare variant of dystrophic epidermolysis bullosa characterized by severely pruritic and cicatricial lesions localized to the extensor extremities. We report a Singaporean Chinese male with epidermolysis bullosa pruriginosa with an underlying ...

Last Updated: 8 Jan 2015

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Case report. Novel and recurrent COL7A1 mutations in Chinese patients with dystrophic epidermolysis bullosa pruriginosa.
 

Author(s): K J Zhu, C Y Zhu, Y Zhou, Y M Fan

Journal:

 

Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr) is a rare subtype of dystrophic epidermolysis bullosa (DEB). This disease is characterized by severe itching, lichenoid nodules or prurigo-like lesions, and linear scarring with a predilection for the extensor limbs. Pathogenic ...

Last Updated: 16 Sep 2014

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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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