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Dystrophic epidermolysis bullosa.

British Journal of Dermatology, 1977
Involvement of the large intestine in a long standing case of dystrophic epidermolysis bullosa was characterized by recurrent episodes of diarrhoea synchronizing with exacerbation of the skin lesions. The radiological investigations revealed two narrow segments, one each in the descending and transverse colon, with ulcer craters in the lower part of ...
V N, Sehgal   +3 more
openaire   +2 more sources

Hand Surgery for Dystrophic Epidermolysis Bullosa

Journal of Pediatric Orthopaedics, 2014
Epidermolysis bullosa (EB) is a group of inherited, mechanobullous disorders caused by mutations in various structural proteins in the skin. The manifestation of these disorders in the hand is of digital contractures and pseudosyndactyly or "cocoon hands," causing significant functional impairment.Our preferred surgical treatment of these patients ...
Shai, Luria   +3 more
openaire   +2 more sources

Fatal cardiomyopathy in dystrophic epidermolysis bullosa

British Journal of Dermatology, 1996
Two unrelated children with autosomal recessive dystrophic epidermolysis bullosa (RDEB) developed fatal dilated cardiomyopathy. Both were malnourished and had severe growth problems. We believe that the most likely cause for the cardiomyopathy is a micronutrient deficiency, most probably selenium deficiency, since the serum selenium level was found to ...
C, Melville   +4 more
openaire   +2 more sources

THE HAND IN RECESSIVE DYSTROPHIC EPIDERMOLYSIS BULLOSA

Hand Clinics, 2000
Recessive dystrophic epidermolysis bullosa is still a difficult challenge for surgeons and doctors. Hand retractions are different from burns. The medical team must have a good knowledge of the disease. Surgery must be performed with the collaboration of every member of the team, as well as the family.
J, Glicenstein, D, Mariani, R, Haddad
openaire   +2 more sources

Gastrostomy and growth in dystrophic epidermolysis bullosa

British Journal of Dermatology, 1996
Chronic malnutrition and growth failure are features of severe dystrophic epidermolysis bullosa (DEB). Conventional dietetic intervention is of limited benefit. Oesophageal dilatation or reconstruction to alleviate stricture is associated with substantial risks.
Haynes, L.   +4 more
openaire   +3 more sources

Epidermolysis bullosa pruriginosa: dystrophic epidermolysis bullosa with distinctive clinicopathological features

British Journal of Dermatology, 1994
We report a study of eight unrelated adult patients with a highly distinctive phenotype of dystrophic epidermolysis bullosa. It is characterized clinically by pruritus, lichenified or nodular prurigo-like lesions, violaceous linear scarring, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and, in some cases, albopapuloid ...
J A, McGrath, O M, Schofield, R A, Eady
openaire   +2 more sources

Epidermolysis Bullosa Nevus in a Patient With Recessive Dystrophic Epidermolysis Bullosa

The American Journal of Dermatopathology, 2014
We present a case of a 6-year-old girl with recessive dystrophic epidermolysis bullosa (EB) who presented with a large pigmented lesion clinically concerning for melanoma. After histological examination and fluorescent in situ hybridization analysis, diagnosis of EB nevus was performed.
Katherine, Fening   +5 more
openaire   +2 more sources

Dystrophic Epidermolysis bullosa inversa: A Case Report

Dermatologica, 2009
The inverse form of recessive dystrophic epidermolysis bullosa is a rare genodermatosis characterized by a smouldering course of integumental blistering with improvement of lesions in adulthood, preferential localizations of lesions in flexural areas, severe oral and esophageal mucosal involvement and nail dystrophy.
G. F. Altomare   +4 more
openaire   +2 more sources

Dystrophic Epidermolysis bullosa in a Family

Dermatologica, 2009
Three cases of the dystrophic type of epidermolysis bullosa in three generations comprising 18 individuals are described. Two of the three cases were females and the other male. The index case was of the vegetans type, while the other two cases were the usual dystrophic type.
V.N. Sehgal   +3 more
openaire   +1 more source

Care of a patient with dystrophic epidermolysis bullosa

Journal of Wound Care, 1995
A report of an improved dressing regime for a young patient with a chronic skin condition
openaire   +2 more sources

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