| Paraneoplastic pemphigus treated with dexamethasone/ cyclophosphamide pulse therapy. | |
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MedLine Citation:
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PMID: 9889426 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Paraneoplastic pemphigus (PNP) is an autoimmune, mucocutaneous bullous disease associated with underlying malignancies. We report a patient with Waldenström's macroglobulinemia who developed clinical, histological and immunopathological features typical of PNP. The patient was treated twice with i.v. dexamethasone and cyclophosphamide pulse therapy (day 1: cyclophosphamide 500 mg i.v.; day 1-3: dexamethasone 100 mg i.v.) at 3-week intervals. Therapy was continued with oral cyclophosphamide (50 mg/d). Two weeks after initiation of treatment, significant improvement of the cutaneous and mucosal lesions was noted. The therapy also had beneficial effects on the macroglobulinemia in terms of a marked reduction of the IgM lambda serum level. Three months after the second pulse, severe stomatitis recurred but the patient rejected any further systemic therapy. The initial response of the usually recalcitrant mucosal and skin lesions of PNP makes dexamethasone/cyclophosphamide pulse therapy an interesting therapeutic option. |
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Authors:
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L R Becker; B C Bastian; U Wesselmann; S Karl; H Hamm; E B Bröcker |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: European journal of dermatology : EJD Volume: 8 ISSN: 1167-1122 ISO Abbreviation: Eur J Dermatol Publication Date: 1998 Dec |
Date Detail:
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Created Date: 1999-02-18 Completed Date: 1999-02-18 Revised Date: 2006-04-24 |
Medline Journal Info:
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Nlm Unique ID: 9206420 Medline TA: Eur J Dermatol Country: FRANCE |
Other Details:
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Languages: eng Pagination: 551-3 Citation Subset: IM |
Affiliation:
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Department of Dermatology, University of Würzburg, Josef-Schneider-Str. 2, D-97080 Würzburg, Germany. becker-lr@mail.uni-wuerzberg.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Administration, Oral Aged Aged, 80 and over Antineoplastic Agents, Alkylating / administration & dosage Antineoplastic Agents, Hormonal / administration & dosage Antineoplastic Combined Chemotherapy Protocols / administration & dosage* Cyclophosphamide / administration & dosage Dexamethasone / administration & dosage Drug Administration Schedule Follow-Up Studies Humans Injections, Intravenous Male Paraneoplastic Syndromes / diagnosis, drug therapy*, etiology Pemphigus / diagnosis, drug therapy*, etiology Treatment Outcome Waldenstrom Macroglobulinemia / complications |
| Chemical | |
Reg. No./Substance:
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0/Antineoplastic Agents, Alkylating; 0/Antineoplastic Agents, Hormonal; 50-02-2/Dexamethasone; 50-18-0/Cyclophosphamide |
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