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Acantholytic epidermolysis bullosa

M D Hoffman1, M G Fleming, R W Pearson

  • 1Department of Dermatology, Rush-Presbyterian-St Luke's Medical Center, Chicago, Ill, USA.

Archives of Dermatology
|May 1, 1995
PubMed
Summary
This summary is machine-generated.

A novel variant of epidermolysis bullosa, termed acantholytic epidermolysis bullosa, is identified. This rare genetic skin disorder presents with specific clinical and histological features.

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Area of Science:

  • Dermatology
  • Genetics
  • Pathology

Background:

  • Describes a new variant of inherited epidermolysis bullosa.
  • Elucidates the clinical, histologic, and ultrastructural features of this condition.

Observation:

  • Autosomal dominant inheritance pattern.
  • Characterized by acral bullae.
  • Histologically demonstrates suprabasal clefting with acantholysis.

Findings:

  • Ultrastructural findings are nonspecific.
  • Findings are reminiscent of benign familial pemphigus.

Implications:

  • Acantholytic epidermolysis bullosa is a rare but distinct clinicopathologic entity.
  • Warrants inclusion in the nosologic classification of epidermolysis bullosa.