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Clinical:
| • | lesions typically first develop in the mouth |
| • | limited to stratified squamous epithelium (only internal organ = proximal esophagus) |
| • | small, flaccid bullae break easily and give rise to large, painful denuded areas |
pemphigus vegetans - probably a clinical variant of p. vulgaris in which chronic lesions develop into hypertrophic vegetative areas
Histology:
| • | suprabasilar split - “row of tombstones” = floor of blister composed of a row of basal keratinocytes |
| • | IgG vs. 130kd adhesion molecule desmoglein 3 (member of cadherin family of adhesion molecules) |
| • | Indirect immunofluorescence (+) in 80-90%; titers parallel disease activity (vs. BP) |
TREATMENT:
prednisone: 1 mg/kg/day tapered slowly over 6-9 months
| • | unresponsive, extensive, or rapidly progressive à 80 to 90mg QD; increase dose by 50% every 4 to 7 days |
Bystrin says:
| • | no such thing as steroid unresponsive disease, only unresponsive to dose |
| • | if respond, will see in less than a week |
cyclophosphamide: 1-2 mg/kg/day or intermittent IV pulse
azathioprine:
IVIg: mechanism = suppresses IgG autoantibody production???
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