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Contributor

Kalpashri Kesavan

Diagnosis

Bart syndrome epidermolysis bullosa epidermolysis bullosa, dermolytic(dystrophic) , dominant aplasia cutis congenita

Body Site

foot ankle

Age

1 days

Pigmentation

light

Organization

confluent

Color

red

Morphology

plaqulous (plaque / nodule / tumor)

Pattern

acral (centripetal - extremity predominant)

Comments

At birth this infant was noted to have thin membranes covering the ankles and feet as well as the top of the left hand. He developed a few additional blisters on his hands and feet during the first 2 days of life, but the new lesions healed quickly. He fed well and developed no blisters on mucous membranes. Immunofluorescent mapping revealed a cleavage plane beneath the basement membrane zone most consistent with a subepidermal blistering condition. The family history was negative and the parents were not related. Four days later the lesions continue to improve with vaseline gauze and gentle cleaning and dressing changes. These findings are most typical of Bart syndrome or dermolytic epidermolysis bullosa with aplasia cutis congenita.

Description

(4 days after birth) large red patches of skin covered with thin reepithelialized skin

Categories

vesiculobullous eruptions, mechanobullous genodermatosis/genetic disorder vesiculobullous eruptions

Image Added

4/4/2010 21:48:32

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Other Resources

PubMed Medline Plus Medscape

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