Author/Editor		Benedičič-Pilih, Ana; Dolenc-Voljč, Mateja; Berčič, Marija; Vizjak, Alenka; Jurčič, Vesna; Kansky, Aleksej
Title		Pemphigus paraneoplasticus
Translated title		Paraneoplastic pemphigus
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 68, št. Suppl 2
Publication year		1999
Volume		str. II-29-33
Language		slo
Abstract		Background. Paraneoplastic pemphigus was defined during the last decade as a new form of the paraneoplastic skin syndrome. It may be observed in association with chronic lymphatic leucoses and lymphomas, with thymomas, Castleman's tumor sarcomas, Waldenstroem's macroglobulinemias as well as with carcinomas. Clinical presentation is not uniform, therefore additional diagnostic procedures are necessary. Methods. A 75-year old female patient with bullous lesions of skin, oral mucosa and conjunctiva that mimicked Pemphigus vulgaris and Erythema multiforme is presented. The skin disease appeared with development of metastases on year after the surgical removal of a rectal adenocarcinoma. Results. Direct immunofluorescence revealed a pattern of immune deposits characteristic for Paraneoplastic pemphigus. By symptomatic treatment with parenteral application of methylprednisolon in high doses, which were gradually descreased, a remission of skin lesions and a partial remission of mucous lesions was achieved. Consluiosns. Five diagnostic criteria for Paraneoplastic pemphigus, including clinical appearance and laboratory findings, are listed.
Summary		Izhodišča. Paraneoplastični pemfigus je v zadnjem desetletju opredeljena nova vrsta paraneoplastičnega sindroma kože, ki se pojavlja pretežno v povezavi s kroničnimi limfatičnimi levkemijami in limfomi, pa tudi timomi, Castlemanovim tumorjem, sarkomi, Waldenstroemovo makroglobulinemijo in karcinomi. Zaradi raznolike klinične slike je ob dejstvu, da gre za paraneoplastični sindrom, pomembna dodatna diagnostika. Metode. Predstavljamo 75-letno bolnico, pri kateri se je ob razvoju zasevkov leto dni po operativni odstranitvi rektalnega adenokarcinoma razvila bulozna dermatoza kože, ustne sluznice in veznic s kliničnimi značilnostmi vulgarnega pemfigusa in multiformnega eritema. Rezultati. Pri naši bolnici smo z direktno imunofluorescenco perilezionalne kože dokazali za paraneoplastični pemfigus tipičen vzorec imunskih depozitov. S simptomatskim zdravljenjem s parenteralno aplikacijo metilprednizolona v visoki začetni dnevni dozi, ki smo jo postopno zniževali, smo dosegli dobro remisijo sprememb na koži in delno na sluznicah. Zaključki. Predstavljamo pet diagnostičnih meril, ki z upoštevanjem klinične slike in laboratorijskih izvidov olajšajo postavljanje diagnoze paraneoplastičnega pemfigusa.
Descriptors		PEMPHIGUS PARANEOPLASTIC SYNDROMES AUTOANTIBODIES FLUORESCENT ANTIBODY TECHNIQUE, DIRECT DESMOSOMES