II. Epidemiology

  1. Incidence: 0.03% of newborns

III. Pathophysiology

  1. Congenital absence of skin
  2. Embryonic defect present in a small subset of patients

IV. Signs

  1. Bulla, ulceration or atrophic lesion

V. Distribution

  1. Most often at the scalp near the vertex

VI. Labs

  1. Biopsy shows absent Dermis or Epidermis

VII. Management

  1. Avoid secondary Bacterial Infection
  2. Surgical excision in some cases

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