Document Detail


Clinical and immunopathological spectrum of paraneoplastic pemphigus.
MedLine Citation:
PMID:  20180886     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Jutta Zimmermann; Friedrich Bahmer; Christian Rose; Detlef Zillikens; Enno Schmidt
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2010-02-18
Journal Detail:
Title:  Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG     Volume:  8     ISSN:  1610-0387     ISO Abbreviation:  J Dtsch Dermatol Ges     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-21     Completed Date:  2010-12-02     Revised Date:  2010-12-27    
Medline Journal Info:
Nlm Unique ID:  101164708     Medline TA:  J Dtsch Dermatol Ges     Country:  Germany    
Other Details:
Languages:  eng; ger     Pagination:  598-606     Citation Subset:  IM    
Affiliation:
Department of Dermatology and Allergology, Bremen Mitte Hospital, Bremen, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Diagnosis, Differential
Female
Humans
Male
Middle Aged
Paraneoplastic Syndromes / diagnosis*,  immunology*
Pemphigus / diagnosis*,  immunology*

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