نتایج جستجو برای: inherited epidermolysis bullosa

تعداد نتایج: 34966  

2017
Guowei Zhao Qing Yang Furen Zhang

Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure's pathogenesis shared some common autoimmune pathways. Moreover, acute blood volume reduction may ...

Journal: :Actas Dermo-Sifiliográficas (English Edition) 2010

Journal: :The Journal of clinical investigation 1982
G P Stricklin H G Welgus E A Bauer

Recessive dystrophic epidermolysis bullosa, a genodermatosis characterized by dermolytic blister formation in response to minor trauma, is characterized by an incresaed collagenase synthesis by skin fibroblasts in culture. Since preliminary studies of partially purified recessive dystrophic epidermolysis bullosa collagenase suggested that the protein itself was aberrant, efforts were made to pu...

Journal: :JPMA. The Journal of the Pakistan Medical Association 2010
Khalid Maudood Siddiqui Sobia Khan

Epidermolysis bullosa is a group of inherited disorders characterized by blistering of the skin as a result of minor trauma. We managed an infant with epidermolysis bullosa undergoing inguinal hernia repair. Anaesthesia was induced with oxygen/nitrous oxide mixture and sevoflurane. Oral tracheal intubation was done with a lubricated laryngoscope blade with KY jelly using atracurium 0.5 mg/kg an...

N Emad Mostofi N Sina R Rafiei R Yaghoobi

Pretibial epidermolysis bullosa is a rare variant of hereditary epidermolysis bullosa characterized by the delayed onset of lesions and their localization. We present two cases, a 35-year-old woman and a 21-year-old man. They clinically had pruritus, nodular prurigo-like or lichenified lesions, violaceous scarring, milia, nail dystrophy and in one case albopapuloid lesions on the trunk. Physica...

Journal: :Anais brasileiros de dermatologia 2012
Hiram Larangeira de Almeida Lísia Nudelmann Nara Moreira Rocha Luis Antonio Suita de Castro

Pasini's albopapuloid epidermolysis bullosa is a very rare subtype of generalized dystrophic dominant epidermolyis bullosa. A 30 year-old white female patient presented since her childhood disseminated small blisters and papules. Light microscopy of a blister showed dermal-epidermal cleavage; moreover, focal areas of dermal-epidermal splitting were also observed. Transmission electron microscop...

2014
Michael J Vanden Oever Jakub Tolar

Epidermolysis bullosa is a group of inherited disorders that can be both systemic and life-threatening. Standard treatments for the most severe forms of this disorder, typically limited to palliative care, are ineffective in reducing the morbidity and mortality due to complications of the disease. Emerging therapies-such as the use of allogeneic cellular therapy, gene therapy, and protein thera...

Journal: :Anais brasileiros de dermatologia 2015
Márcia Carolline dos Santos Sousa Carmen Dea Ribeiro de Paula Pedro Luiz Tauil Izelda Maria Carvalho Costa

Epidermolysis bullosa comprises a group of phenotypically different genodermatosis, hereditary or acquired, characterized by skin fragility and subsequent formation of blisters in response to mechanical trauma, and which may also affect mucous membranes. This study aimed to analyze the relation between the nutritional, hematologic, infectious characteristics and the type of epidermolysis bullos...

2013
Claudia R. Albornoz Jane Goldstein Geoffrey E. Hespe Virgilio Sacchini Evan Matros

OBJECTIVES Epidermolysis bullosa is a rare skin disorder characterized by blister formation in response to minor trauma as well as extracutaneous manifestations. Details of the surgical history and technical considerations for performing breast reconstruction in a patient with epidermolysis bullosa are discussed. METHOD The history and details of breast reconstruction in a patient with epider...

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