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Pemphigus vulgaris

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Pemphigus vulgaris

  1. 1. Pemphigus Vulgaris
  2. 2. What is it? <ul><li>This patient complains of this painful outbreak. You tell them they have </li></ul><ul><li>A) Poor hygiene </li></ul><ul><li>B) Bullous Pemphigoid </li></ul><ul><li>C) Pemphigus Vulgaris </li></ul><ul><li>D) Bullous Impetigo </li></ul>
  3. 3. Pemphigus Vulgaris <ul><li>Pemphigus vulgaris is the most common form of pemphigus </li></ul><ul><li>Oral lesions usually precede the skin blisters by weeks to months (80% present with oral lesions first) </li></ul>
  4. 4. Pemphigus Vulgaris <ul><li>The primary lesion is a flaccid blister/bullae that easily ruptures, leaving erosions and crusting, and eventual marked post-inflammatory changes </li></ul>
  5. 5. Pemphigus Vulgaris <ul><li>In severe cases the oral epithelium is completely denuded and is associated with intraoral pain that is particularly worsened by eating </li></ul><ul><li>Infrequently other mucous membranes maybe involved (ocular and genitourinary) </li></ul>
  6. 6. Pemphigus Vulgaris <ul><li>Nonpruritic skin blisters varying in size from 1 to several cm’s gradually appear and maybe localized for a considerable time </li></ul>
  7. 7. Pemphigus Vulgaris <ul><li>The lesions become invariably more generalized if left untreated. </li></ul><ul><li>It is usually most accentuated in the intertriginous areas </li></ul>
  8. 8. Pemphigus Vulgaris <ul><li>The etiology is from autoimmunity to the pemphigus vulgaris antigen (desmoglein 3) a member of the cadherin family and a normal component of human keratinocyte cell membranes </li></ul>
  9. 9. Pemphigus Vulgaris <ul><li>DIF reveals IgG in the intercellular regions of the epidermis in and around the affected parts of the skin or mucous membranes </li></ul><ul><li>C3, IgM and IgA are found much less frequently </li></ul><ul><li>Indirect IF studies are positive and can be used as an indicator of disease activity (monkey esophagus) </li></ul>
  10. 10. Pemphigus Vulgaris <ul><li>The basal cells lose their intercellular bridges but they remain attached to the dermis, giving a ‘tombstone appearance’ </li></ul><ul><li>The blister cavity usually contains a few acantholytic cells which often show degenerative changes </li></ul>
  11. 11. Diagnosis <ul><li>Clinical picture </li></ul><ul><li>Skin biopsy for light microscopy </li></ul><ul><li>Skin biopsy for direct IF studies </li></ul><ul><li>Indirect IF studies (levels correlate with disease activity) </li></ul>
  12. 12. Pemphigus Vegetans <ul><li>This is a variant of P. Vulgaris in which markedly hyperplastic erosive plaques develop, primarily in intertriginous regions (groin and axillary vaults) </li></ul><ul><li>At times, such lesions occur in some patients with longstanding pemphigus vulgaris </li></ul>
  13. 13. Pemphigus Vegetans <ul><li>P. Vegetans is characterized by flaccid bullae that become erosions and form fungoid vegetations or papillomatous proliferations especially in the body folds </li></ul><ul><li>The bullae rupture and become exuberant with verrucous vegetations, capped by crusts and surrounded by a zone of inflammation </li></ul>
  14. 14. Pemphigus <ul><li>Other variants: </li></ul><ul><li>P. erythematosus </li></ul><ul><li>P. Foliaceous </li></ul><ul><li>P. Vulgaris </li></ul><ul><li>P. Vegetans </li></ul>
  15. 15. P. Vulgaris Treatment <ul><li>Oral steroids </li></ul><ul><li>Azathioprine </li></ul><ul><li>Cyclosporine </li></ul><ul><li>Plasmapheresis </li></ul><ul><li>Rituximab </li></ul>