| • | a rare variant of pemphigus vulgaris |
| • | characterized by vegetating erosions, primarily in the flexures |
| • | 2 subtypes may be considered to form a clinical spectrum from the severe Neumann type to the mild Hallopeau type |
pathogenesis: (both types)
| • | antibodies react with the 130 kDa pemphigus vulgaris antigen (i.e. desmoglein 3) |
| • | and possibly other antigens |
| • | antibodies in the Hallopeau type also react with desmocollins I and II |
clinical:
| • | involvement of the oral mucosa is almost invariable |
| • | lesions are primarily flexural |
| • | vegetations studded with pustules |
ddx: (of vegetating, pustular lesions in the flexures)
ddx: (histologically)
(these conditions have distinctive clinical features in addition to negative immunofluorescent studies)
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