Pemphigus may occur as a paraneoplastic syndrome. Camisa and Helm proposed criteria for its diagnosis.


Major criteria:

(1) polymorphous mucocutaneous eruption

(2) presence of a concurrent internal neoplasia

(3) characteristic serum immunoprecipitation findings (presence of autoantibodies)


Minor criteria:

(1) positive cytoplasmic staining of rat bladder epithelium by indirect immunofluorescence

(2) intercellular and basement membrane zone immunoreactants on direct immunofluorescence of perilesional tissue

(3) acantholysis in biopsy specimens from at least one anatomic site of involvement



• Immunoprecipitation findings include demonstration of autoantibodies to desmoplakins I and/or II, which are the major bullous pemphigoid antigens.

• Positive staining of rat bladder epithelium is reported as being 76% sensitive and over 90% specific.



• The combinations of major and minor criteria required to make the diagnosis are not listed.


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