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Clinical and immunopathological spectrum of paraneoplastic pemphigus
Author(s) -
Zimmermann Jutta,
Bahmer Friedrich,
Rose Christian,
Zillikens Detlef,
Schmidt Enno
Publication year - 2010
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/j.1610-0387.2010.07380.x
Subject(s) - paraneoplastic pemphigus , autoantibody , medicine , malignancy , dermatology , pemphigus , immunology , pathology , antibody
Summary Background: Paraneoplastic pemphigus (PNP) is a rare life‐threatening autoimmune bullous disorder. The clinical picture is often polymorphous and blisters may be absent. Autoantibodies are directed against several target antigens, including plakins and desmogleins. PNP has a high mortality rate that does not directly depend on the malignancy of the neoplasm. No generally accepted diagnostic criteria for PNP have been defined. Patients and Methods: On the basis of four selected PNP patients and the relevant literature, the spectrum of clinical and immunopathological findings as well as pathogenesis and treatment options of PNP are presented. Results: In addition to a neoplasm, severe stomatitis and the presence of autoantibodies against periplakin and envoplakin are characteristic for PNP. Based on the presented data, diagnostic criteria are proposed. Conclusions: Knowledge of the polymorphous clinical picture and the complex autoantibody response is essential for an early diagnosis of PNP which has implications for both prognosis and rapid initiation of treatment.