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Issue 1107 coverAutoimmunity, Part C The Mosaic of Autoimmunity Volume 1107 published June 2007
Ann. N.Y. Acad. Sci. 1107: 231–238 (2007). doi: 10.1196/annals.1381.025
Copyright © 2007 by the New York Academy of Sciences
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Part III. Autoimmunity and Cancer

Paraneoplastic Pemphigus Associated with Castleman's Disease

Usefulness of the Laboratory of Autoimmunity in the Diagnosis of This Disease

I. ALARCÓN-TORRESa, J. BASTIDA-IÑARREAb, M.J. RODRÍGUEZ-SALIDOb, J. GÓMEZ-DUASOb, I. RUA-FIGUEROAc, G.D. GARCÍA-AGUILARa AND R. MARTÍN-ALFAROa

a Clinical Biochemistry Department, Laboratory of Autoimmunity, University Hospital of Gran Canaria "Doctor Negrín," Las Palmas de Gran Canaria, Spain b Dermatology Service, University Hospital of Gran Canaria "Doctor Negrín," Las Palmas de Gran Canaria, Spain c Rheumatology Service, University Hospital of Gran Canaria "Doctor Negrín," Las Palmas de Gran Canaria, Spain

Key Words: paraneoplastic pemphigus • autoimmunity • indirect immunofluorescence test • immunoprecipitation • desmoplakin • desmoglein • Castleman's disease

Address for correspondence: Inmaculada Alarcón-Torres, Clinical Biochemistry Department, University Hospital "Doctor Negrín," Barranco de la Ballena s.n. 35010, Las Palmas de Gran Canaria, Spain. Voice: +0034928449500; fax: +0034928450089.  ialator{at}gobiernodecanarias.org

We report on a case of paraneoplastic pemphigus associated with Castleman's disease. Clinical pathologic features were not conclusive. Diagnosis was established thanks to the detection of seric autoantibodies directed against intercellular substance by indirect immunofluorescence on monkey esophagus. The positive result of this test prompted us to reevaluate the patient and to detect the occult neoplasia. The demonstration of autoantibodies against plakins is the key marker of this disease but depends on tests that may not be readily available in many places like immunoprecipitation, immunoblotting, or indirect immunofluorescence over rat bladder. In this setting, tests like indirect immunofluorescence over monkey esophagus, although unspecific, may aid in reaching the appropriate diagnosis. This case illustrates the importance of the laboratory of autoimmunity in the diagnosis of this type of pemphigus.






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