| • | unknown etiology; (good Archives article Feb 2001) |
| • | associated with underlying neoplasm (most commonly: lymphoreticular – especially non-Hodgkin’s lymphoma and CLL) |
| • | clinical – polymorphous eruption resembling erythema multiforme and typically affects mucosal surfaces and palms and soles |
| • | intractable stomatitis = the one feature that is persistent and resistant to therapy |
| • | multiple antigens – desmoplakin 1 (250kd), BP1 (230kd), desmoplakin 3 (210kd) |
| • | serum for IIF on rodent bladder substrate (PP patients have IgG which bind to all desmosome containing epithelium, not just to stratified squamous epithelia as in PV) |
|