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Herpetiform Pemphigus

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IgG4 to Dsg1> Dsg3, possibly weaker antibody than in PV

  • Urticarial plaques and vesicles in herpetiform distribution
  • Less severe and more chronic than PV
  • May have features of PF (75%) or PV (25%) during the course

Similar approach to PnP:

  • H & E (see subsection): Eosinophilic spongiosis and subcorneal pustules, little to no acantholysis
  • DIF: IgG4 Dsg1 > Dsg3

  • Similar approach to above.
  • Often first line is Sulfasalazine (sulfones).

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