Paraneoplastic Pemphigus

Although an occasional association between pemphigus and malignancy had been recognised for many years, it was not until 1990 that paraneoplastic pemphigus was recognised as a distinct clinical, histological and im-munocytochemical entity [5]. The condition is seen predominantly in association with B-cell lymphoprolif-erative disorders, especially non-Hodgkin lymphoma, chronic lymphocytic leukaemia, Castleman disease, thymoma and Waldenstrom macroglobulinaemia [92]. Less commonly it is associated with non-lymphoid neoplasms, including some carcinomas of the bronchus, breast and pancreas. In some cases showing otherwise typical features of the disease, no underlying malignancy is found. Paraneoplastic pemphigus is characterised by the following features:

■ Painful mucocutaneous vesiculo-bullous eruptions;

■ Histopathologic features of intraepithelial acantholy-sis and vacuolar interface changes;

■ Demonstration of intercellular epithelial IgG and C3, with or without granular linear deposition of complement along the BMZ;

■ Presence of circulating autoantibodies that bind to the surfaces of stratified squamous epithelia as well as simple, columnar and transitional epithelia;

■ Presence of a characteristic complex of proteins derived from keratinocytes and serum antibodies demonstrated by serum immunoprecipitation. These include desmoplakins I and II, bullous pemphigoid antigen I, envoplakin and periplakin [92].

In addition, paraneoplastic pemphigus tends to be extremely refractory to the usual immunosuppressant drugs used to control pemphigus vulgaris.

Paraneoplastic pemphigus is most common between the ages of 45 and 70 years and there appears to be a male predominance. The mouth is almost always involved and oral lesions present as a painful, intractable stomatitis that extends into the oropharynx and often beyond the vermilion borders of the lips. It causes blisters and irregular, ragged ulceration. The buccal mucosa and lips are the most common sites, but almost anywhere in the mouth, oropharynx and nasopharynx can be involved. About two-thirds of patients have conjunctival involvement characterised by frequently severe pseudomembranous conjunctivitis and sym-blepharon.

Microscopy shows intraepithelial acantholysis with suprabasal clefting, dyskeratotic keratinocytes, basal cell liquefaction, and epithelial inflammatory cell exocytosis [78]. In many cases, however, the condition cannot be distinguished from conventional pemphigus [89]. The overall appearances suggest that there is an overlap between paraneoplastic pemphigus and erythema multiforme. Indirect immunofluorescence on the transitional epithelium of rat bladder appears to be a highly specific test for paraneoplastic pemphigus [100].

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