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Howard County Hospital Public Relations

Diagnosis

Bart syndrome epidermolysis bullosa, dermolytic (dystrophic) epidermolysis bullosa, dermolytic(dystrophic) , dominant epidermolysis bullosa

Body Site

foot

Age

1 days

Pigmentation

light

Organization

confluent

Color

red

Morphology

depressed (atrophy, loss of soft tissue,scar)

Pattern

symmetric acral (centripetal - extremity predominant)

Comments

At birth this vigorous boy was noted to have atrophic plaques on the ankles. Erosions subsequently developed with minor trauma, and the lesions were managed with topical antibiotic ointment and non-stick dressings. By 2 weeks of age the plaques had shrunk by 50 percent . At birth he also had an intact blister on the left middle finger which promptly dried and peeled. Although he developed small vesicles on the lips, they did not interfete with feeding, and no lesions appeared in the mouth. He weighed 7 pounds, 11 ounces at birth and gained 5 ounces by 2 weeks of age. His mother and and a maternal nephew had a similar history of neonatal and early childhood acral blsitering with partial loss of finger and toe nails and scarring of the hands and feet. Bart syndrome represents a dominant dermolytic variant of epidermolysis bullosa.

Description

symmetric well demarcated atrophic plaques covered with thin epithelium

Categories

genodermatosis/genetic disorder vesiculobullous eruptions vesiculobullous eruptions, mechanobullous

Image Added

12/1/2004 22:49:53

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PubMed Medline Plus Medscape

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