Results 171 to 180 of about 2,763 (196)

Junctional epidermolysis bullosa

1990
In 1935, Herlitz [1] recognized the existence of a distinct hereditary blistering disease that was lethal in early infancy. Since then, the ‘hereditary lethal’ form of epidermolysis bullosa (EB) has borne his name, but there was no certain means of delineating Herlitz disease from the more common dystrophic forms, which could be clinically confusing ...
Robin A. J. Eady, Michael J. Tidman
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Non-lethal junctional epidermolysis bullosa in a dog

British Journal of Dermatology, 1997
We report a 4-year-old female mongrel dog with a history since birth of erosions and atrophic skin, with pigmentation and alopecia on the face, trunk and extremities, together with dystrophic nails. Light microscopy revealed subepidermal blisters with minimal inflammation and electron microscopy confirmed that the ultrastructural site of separation ...
M, Nagata, T, Iwasaki, H, Masuda, H, Shimizu   +3 more
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Acute Laryngeal Obstruction in Junctional Epidermolysis Bullosa

Pediatric Dermatology, 1987
Abstract: A boy with junctional epidermolysis bullosa died from acute laryngeal obstruction at the age of 29 months, having been hoarse since early infancy. Post mortem studies showed gross narrowing of the laryngeal airway by cystic dilatations of the ducts of the seromucinous glands, and replacement of the laryngeal epithelium by oedematous ...
H, Davies, D J, Atherton
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Dermal eosinophilic infiltrate in junctional epidermolysis bullosa

Journal of Cutaneous Pathology, 2015
Junctional epidermolysis bullosa (JEB) is a rare genodermatosis characterized by a split in the lamina lucida usually because of mutations in LAMA3, LAMB3 and LAMC2 resulting in absence or reduction of laminin‐332. Rare subtypes of JEB have mutations in COL17A1, ITGB4, ITGA6 and ITGA3 leading to reduction or dysfunction of collagen XVII, integrin α6β4 ...
Ami, Saraiya, Catherine S, Yang, Jinah, Kim, Lionel, Bercovitch, Leslie, Robinson-Bostom, Gladys, Telang   +5 more
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Treatment of junctional epidermolysis bullosa with epidermal autografts

Journal of the American Academy of Dermatology, 1987
We have successfully treated chronic facial erosions in three boys with junctional epidermolysis bullosa. In each patient, keratinocytes were harvested from the roof of suction blisters created on clinically uninvolved skin. They were grown in tissue culture on collagen sponges and grafted onto facial erosions that were previously treated with 2 ...
D M, Carter, A N, Lin, M C, Varghese, D, Caldwell, L A, Pratt, M, Eisinger   +5 more
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Epidermolysis bullosa hereditaria with Junctional Blistering in an Adult

Dermatologica, 2009
A 38-year-old patient with epidermolysis bullosa is described, in whom junctional blister formation is revealed by electron microscopy. Clinical and ultrastructural differences from the recessive dystrophic type (Hallopeau-Siemens) and from the lethal type (Herlitz) of epidermolysis bullosa are discussed in detail.
I, Hashimoto, U W, Schnyder, I, Anton-Lamprecht   +2 more
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Junctional Forms of Epidermolysis Bullosa

1998
The group of heritable genodermatoses known as epidermolysis bullosa (EB) are characterized by blistering and skin and mucous membrane fragility in response to mechanical trauma. EB is divided into three major categories based on the level of tissue separation, as determined by transmission electron microscopy.
Angela M. Christiano, Jouni Uitto
openaire   +1 more source

Ultrastructure of junctional epidermolysis bullosa in Belgian foals

Journal of Comparative Pathology, 1988
Ultrastructural examination of a mechanobullous disease of probable hereditary nature in Belgian foals, confirmed light microscopic findings that separation of the dermo-epidermal junction occurred through the lamina lucida of the basement membrane, leaving the intact lamina densa adherent to the dermis and the plasmalemma of the basal epithelial cells
G C, Johnson, C W, Kohn, C W, Johnson, F, Garry, D, Scott, S, Martin   +5 more
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Junctional Epidermolysis Bullosa in a Young Patient

Journal of Pediatric Ophthalmology & Strabismus, 2003
Inci, Irak, Stphen M, Soll, Jeanette M, Camacho   +2 more
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Leading edge: emerging drug, cell, and gene therapies for junctional epidermolysis bullosa

Expert Opinion on Biological Therapy, 2020
Allison R Keith, Christen L Ebens, Jakub Tolar   +2 more
exaly