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Information

  1. Epidermolysis bullosa is characterized by abnormal collagen that is insufficient for anchoring skin layers to each other. (Laryngeal involvement is rare.)
    1. Pressure applied perpendicular to the skin is less likely to produce separation of skin layers (intradermal fluid accumulation and bullae formation) than are lateral shearing forces.
    2. Management of anesthesia is based on avoidance of trauma to the skin and mucous membranes from adhesive tape, blood pressure cuffs, tourniquets, and adhesive electrodes.
      1. Lubrication of the face mask is useful for decreasing trauma to the face.
      2. Use of upper airway instruments and passage of an esophageal stethoscope should be avoided. The safety of tracheal intubation has been established for patients with the dystrophic form of this disease.
      3. Ketamine is useful anesthesia for superficial surgical procedures.
  2. Pemphigus is an autoimmune vesiculobullous disease that involves the skin and mucous membranes. Oral lesions are common, and corticosteroids are effective in therapy.

Outline

Rare Coexisting Diseases

  1. Musculoskeletal Diseases
  2. The Myotonias
  3. Familial Periodic Paralysis
  4. Myasthenia Gravis
  5. Guillain-Barré Syndrome (Polyaradiculoneuritis)
  6. Central Nervous System Diseases
  7. Inherited Disorders
  8. Anemias
  9. Collagen Vascular Diseases
  10. Skin Disorders