Pretibial epidermolysis bullosa. Successful therapy with a skin graft

Furue, Masutaka

doi:10.1001/archderm.122.3.310

Cited by 17 publications

(12 citation statements)

References 8 publications

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“…Pretibial DDEB , as the name implies, almost exclusively involves the anterior lower legs [52,53]. Individual lesions, which tend to be papular or plaque-like, are oftentimes somewhat violaceous, suggesting the clinical diagnosis of lichen planus.…”

Section: Clinical Descriptionmentioning

confidence: 99%

Inherited epidermolysis bullosa

Fine

2010

Orphanet J Rare Dis

222

193

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Inherited epidermolysis bullosa (EB) encompasses a number of disorders characterized by recurrent blister formation as the result of structural fragility within the skin and selected other tissues. All types and subtypes of EB are rare; the overall incidence and prevalence of the disease within the United States is approximately 19 per one million live births and 8 per one million population, respectively. Clinical manifestations range widely, from localized blistering of the hands and feet to generalized blistering of the skin and oral cavity, and injury to many internal organs. Each EB subtype is known to arise from mutations within the genes encoding for several different proteins, each of which is intimately involved in the maintenance of keratinocyte structural stability or adhesion of the keratinocyte to the underlying dermis. EB is best diagnosed and subclassified by the collective findings obtained via detailed personal and family history, in concert with the results of immunofluorescence antigenic mapping, transmission electron microscopy, and in some cases, by DNA analysis. Optimal patient management requires a multidisciplinary approach, and revolves around the protection of susceptible tissues against trauma, use of sophisticated wound care dressings, aggressive nutritional support, and early medical or surgical interventions to correct whenever possible the extracutaneous complications. Prognosis varies considerably and is based on both EB subtype and the overall health of the patient.

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Section: Clinical Descriptionmentioning

confidence: 99%

Inherited epidermolysis bullosa

Fine

2010

Orphanet J Rare Dis

222

193

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“…It may be reasonable to assume that, in this case also, repeated vesicle formation and epithelization had served as a trigger for the development of angiokeratoma (5,6). Although histological examination of the vesicles could not be performed because vesicles had ceased, a diagnosis of pretibial epidermolysis bullosa (7,8) seems highly probable, based on the findings of her younger brother. This interesting association of pretibial epidermolysis bullosa with solitary angiokeratoma, although rare, prompted me to report this case.…”

Section: Commentmentioning

confidence: 85%

Solitary Angiokeratoma Seen in a Patient with Pretibial Epidermolysis Bullosa

Maeda

1987

The Journal of Dermatology

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Solitary angiokeratoma was observed on the right leg of a 50‐year‐old Japanese female. In her childhood, she had repeatedly developed vesicles on the legs, and a possible diagnosis of pretibial epidermolysis bullosa was made, based on additional findings. Clinically, the condition resembled malignant melanoma and basal cell epithelioma. Possibly repeated injuries predisposed her to the development of solitary angiokeratoma.

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“…In the past, tissue-engineered artificial allografts, 4,5 cultured allografts, 3,6 cultured autografts, 7,8 and full-thickness or split-thickness autografts 9,10 have been successfully applied in patients with EB. In a Cochrane review concerning venous leg ulcers in a non-EB population, no differences in efficacy were seen in the different types of skin grafting.…”

Section: Discussionmentioning

confidence: 99%