PEMPHIGUS  VULGARIS (PV)

 

Etiology:

  1. Chronic blistering disorder of skin and mucous membranes caused by  IgG autoantibodies against a cell adhesion molecule located in the epidermis.

S & S:

  1. Oral lesions are very common in PV and generally precede the skin bullae by weeks to months. They are tender and cause dysphagia.  Involvement of the proximal esophagus may cause substernal chest pain and dyspepsia.
     
  2. Flaccid bullae appear on the face, neck, and trunk.  They are very fragile and are most often  unroofed at time of presentation leaving a shallow erosion.
     
  3. An intact bullae will show the  Nikolsky sign (bullae extends along the edge with gentle pressure to center). 

DX:

  1. Skin biopsy reveals an intraepidermal cleft and direct immunoflourescence of  biopsy specimen shows IgG bound to the cell surface.
     
  2. Serum titers are used for indirect immunoflourescent evaluation to confirm the presence of circulating autoantibodies.

Treatment:

  1. Prednisone 1 mg/kg/day tapered slowly over 6 to 9 months.
     
  2. For resistant disease or if steroids are contraindicated, use other  immunosuppressants (azathioprine, cyclosphosphamide, chlorambucil, methotrexate or gold).

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