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IGA epidermolysis bullosa acquisita during pregnancy
ESDR I JSID I SID Abstracts
0265 lMM"NOLOGlCAL CHARACTERIZATION
OF A UNtQUE FOCUS OF ENDEMtC PEMPHIGUS FOLIACEUS IN THE RURAL AREA OF EL BAGRE, COLOMEIA. u A&u. Juan G. Maldonado, Andres Jaramillo. Pablo J Paliflo. St& Prada. Walter Leon anrj Mwlova Fernando. Biomed&, Basic Sciencs Caporetion, “nivers~ d A,&qua. -,,i”, Color&a A case-control siudy was designed to characterize some lmmunologtcal aspects in one locus of endemic pemphigus foliaceous (EPF) I” El Bagre ColomMa Previously we demonstrafed that this focus differs in son~e clInical. epidemiological and environmental risk factors from others EPF foci. Forty-two patients and 27 controls were studied whom matched by age, sex and work condition. The presence of autoantibodies In the seem and sktn of EPF patients were analyzed by direct and indirect immunofluorescence @IF. IIF). immunoblotting, immurwprecipitatton (IP) and ELISA Dy IIF. lgG4 was the commonest IgG subclass producing intercellular staining between keratinocytes. Using anti-lgG3 subclass, an intracelkrlar imnlunofluorescence was detected in 62 2% of the cases and in 6.6% of the controls (~~005) In skin lesions from 18 EPF patient., DIF showed an intercellular keratinocyte staining with predominating IgGl and lgG4 subclasses and C3. In 62.7% of EPF patients. IIF revealed a basal membrane zone staining with IgM. C3 a IgG In a linear or granular pattern. By immunoblottlng. using ncfmal human skin as a substtak?. 33% of EPF sara showed reactivity with a 160 kDa band Moreover, 40% of EPF and 37% of control sera from El Bagre showed reactivity against two bands of approxmv&4y 210 and 195 kDa These bands were “ot detected I” othar controls living out from the endemic area On the other hand 93.3 % of EPF sara immunopreapitated a 46 kDa peptide obtained by trypsinlzaUon and Cor+A afflnlty chromatography from bovine epidermis. An ELISA assay performed using the Con-A affinity pwified extract. showed a high correlalionshlp between IP and ELISA resuiis. According lo clinical. epidemiological and these finding we suggest that the EPF from EB is other variety that belongs to the big syndrome of pemphigus.
0266
0269
0267
0270
AUTOANTlBODlES FROM PATIENTS WITH CICATRICIAL PEMPHIGOID RECOGNIZE LAMININ a3 CHAIN MONOMER. Chihiro Matsui. Tafo Kitaaawa, Minoru Ohtsuvama, Masaaki Morohashi Warren k Hoeffler*. Department of Dermatology, Toyama Medical and Pharmaceutical University, Toyama, JAPAN, * Department of Dermatology, Stanford University, Stanford, USA Recent studies have identified a group of cicatricial pemphigoid patients who have IgG anti-basement membrane autoantibodies that recognize epiligrin, a heterotrimeric protein closely related or identical to laminin 5. To further understand the pathophysiology of blister formation in these patients, we have sought to identify the specific polypeptide(s) and epitope(s) targeted by their auto antibodies. Comparative studies show that sera from these patients and polyclonal antibody immunoprecipitate the same set of disulfide-linked polypeptides from media and cell fraction of biosynthetically radiolabeled human keratinocytes. Moreover, these sera immunoprecipitate a3 subunit monomer of laminin 5 from keratinocyte cell fraction but show no reactivity to 83 and y2 monomers. The same sera precipitate same polypeptides from mucous membrane-derived keratinocytes. The epitope mapping using bacterial fusion protein of a3 subunit is now in progress.
HISTOPATHOLOGICAL FEATURES AND MERCURY DEPOSITS IN SKIN BIOPSIES FROM ENDEMIC PEMPHIGUS FOLIACEUS (EPF) PATIENTS FROM THE RURAL AREA OF EL BAGRE. COLOMBIA Leon Warier. Ana M. Aixeu. G”mar Warfuinrre and Fernan,& f&&& University 0, A,rficqlaa. Cobmbia am, Lund “&‘a~-&,,, Sweden. A case-control study ot an endemic focus of pemplugus foliaceus was carried out. Forty-two patients and 27 controls were tested. All patients ware linked v&h mininpl farming aottwties Sun exposure was the biggest enwcnmentat
factor ass&&d
to all patients.
Hair mernny
detected and serec IgE were found Mgher m patients (pcO.05). Anhough the EPF bagnosis was made by clinical. epidemiological and immunological criteria, tistopathotogy using H 8 E stain rweated an hetemgenews p~dure In 42.3% pemphigus foliaceus, in 23% chronic dermatitis. in 15 4% pusttutar dermatitis, m 11 5% lupus. in 7.7% psodaeiform dermatitis and in 3.3% Darter’s Ike-disease We detected acanthosis in gl%. hyperpara-keratosis m 81%. acantholysls in 50%. spongloas m 17%. atrophy in 1296. epidemtal pustules or vesicle pustules in 37%. hyperpigmentation at the SMZ in 40%. active metanocytes in 50%. Llquefachon of the Ed&Z in 29%. melanophages in 97%. papitlomatosis in 37%. enlargement, dilatabon, tortutity an mcnase in the waits from the Mood vessel in 95% were found In dermis hislkxytes and lymphocytes in 95%. polymorpho nuclear in 85%. eosnophds in 39%. and plasmabc ceils in ?@A, scmc patchy inri.trates of lynphoid c&s mlinlY arotmd blood vessels were also observed. In d&s deposds of fibrin, dermat coltagenization and edema were detected EpiderrrIal appendages. rweals presence of Demodex in 12% of patients and in 7 1% of controls. In 40 % of the patients focal necrosis of sweat glands aocompanied for spongioeis of the acmsidngium, hypedrophy. hyperptasia and cellular infiltration were visualred By atiometalograpy technique, traces of mercury were detected in these glands. In co”tr& from El Sagre mercuy was sfso detected I” 40 %. bti without sweat glands necmss Based on these ~~11s and in the occupational exposure of EPF patients (sun and mercury), we suggest that these can play b r& for tngger EPF disease m EL Bagre. .&so the histopathotogicat aiterations of the SMZ, and necrosis of the w.eat glands are umque to this focus.
0265 lMM"NOLOGlCAL CHARACTERIZATION
OF A UNtQUE FOCUS OF ENDEMtC PEMPHIGUS FOLIACEUS IN THE RURAL AREA OF EL BAGRE, COLOMEIA. u A&u. Juan G. Maldonado, Andres Jaramillo. Pablo J Paliflo. St& Prada. Walter Leon anrj Mwlova Fernando. Biomed&, Basic Sciencs Caporetion, “nivers~ d A,&qua. -,,i”, Color&a A case-control siudy was designed to characterize some lmmunologtcal aspects in one locus of endemic pemphigus foliaceous (EPF) I” El Bagre ColomMa Previously we demonstrafed that this focus differs in son~e clInical. epidemiological and environmental risk factors from others EPF foci. Forty-two patients and 27 controls were studied whom matched by age, sex and work condition. The presence of autoantibodies In the seem and sktn of EPF patients were analyzed by direct and indirect immunofluorescence @IF. IIF). immunoblotting, immurwprecipitatton (IP) and ELISA Dy IIF. lgG4 was the commonest IgG subclass producing intercellular staining between keratinocytes. Using anti-lgG3 subclass, an intracelkrlar imnlunofluorescence was detected in 62 2% of the cases and in 6.6% of the controls (~~005) In skin lesions from 18 EPF patient., DIF showed an intercellular keratinocyte staining with predominating IgGl and lgG4 subclasses and C3. In 62.7% of EPF patients. IIF revealed a basal membrane zone staining with IgM. C3 a IgG In a linear or granular pattern. By immunoblottlng. using ncfmal human skin as a substtak?. 33% of EPF sara showed reactivity with a 160 kDa band Moreover, 40% of EPF and 37% of control sera from El Bagre showed reactivity against two bands of approxmv&4y 210 and 195 kDa These bands were “ot detected I” othar controls living out from the endemic area On the other hand 93.3 % of EPF sara immunopreapitated a 46 kDa peptide obtained by trypsinlzaUon and Cor+A afflnlty chromatography from bovine epidermis. An ELISA assay performed using the Con-A affinity pwified extract. showed a high correlalionshlp between IP and ELISA resuiis. According lo clinical. epidemiological and these finding we suggest that the EPF from EB is other variety that belongs to the big syndrome of pemphigus.
0266
0269
0267
0270
AUTOANTlBODlES FROM PATIENTS WITH CICATRICIAL PEMPHIGOID RECOGNIZE LAMININ a3 CHAIN MONOMER. Chihiro Matsui. Tafo Kitaaawa, Minoru Ohtsuvama, Masaaki Morohashi Warren k Hoeffler*. Department of Dermatology, Toyama Medical and Pharmaceutical University, Toyama, JAPAN, * Department of Dermatology, Stanford University, Stanford, USA Recent studies have identified a group of cicatricial pemphigoid patients who have IgG anti-basement membrane autoantibodies that recognize epiligrin, a heterotrimeric protein closely related or identical to laminin 5. To further understand the pathophysiology of blister formation in these patients, we have sought to identify the specific polypeptide(s) and epitope(s) targeted by their auto antibodies. Comparative studies show that sera from these patients and polyclonal antibody immunoprecipitate the same set of disulfide-linked polypeptides from media and cell fraction of biosynthetically radiolabeled human keratinocytes. Moreover, these sera immunoprecipitate a3 subunit monomer of laminin 5 from keratinocyte cell fraction but show no reactivity to 83 and y2 monomers. The same sera precipitate same polypeptides from mucous membrane-derived keratinocytes. The epitope mapping using bacterial fusion protein of a3 subunit is now in progress.
HISTOPATHOLOGICAL FEATURES AND MERCURY DEPOSITS IN SKIN BIOPSIES FROM ENDEMIC PEMPHIGUS FOLIACEUS (EPF) PATIENTS FROM THE RURAL AREA OF EL BAGRE. COLOMBIA Leon Warier. Ana M. Aixeu. G”mar Warfuinrre and Fernan,& f&&& University 0, A,rficqlaa. Cobmbia am, Lund “&‘a~-&,,, Sweden. A case-control study ot an endemic focus of pemplugus foliaceus was carried out. Forty-two patients and 27 controls were tested. All patients ware linked v&h mininpl farming aottwties Sun exposure was the biggest enwcnmentat
factor ass&&d
to all patients.
Hair mernny
detected and serec IgE were found Mgher m patients (pcO.05). Anhough the EPF bagnosis was made by clinical. epidemiological and immunological criteria, tistopathotogy using H 8 E stain rweated an hetemgenews p~dure In 42.3% pemphigus foliaceus, in 23% chronic dermatitis. in 15 4% pusttutar dermatitis, m 11 5% lupus. in 7.7% psodaeiform dermatitis and in 3.3% Darter’s Ike-disease We detected acanthosis in gl%. hyperpara-keratosis m 81%. acantholysls in 50%. spongloas m 17%. atrophy in 1296. epidemtal pustules or vesicle pustules in 37%. hyperpigmentation at the SMZ in 40%. active metanocytes in 50%. Llquefachon of the Ed&Z in 29%. melanophages in 97%. papitlomatosis in 37%. enlargement, dilatabon, tortutity an mcnase in the waits from the Mood vessel in 95% were found In dermis hislkxytes and lymphocytes in 95%. polymorpho nuclear in 85%. eosnophds in 39%. and plasmabc ceils in ?@A, scmc patchy inri.trates of lynphoid c&s mlinlY arotmd blood vessels were also observed. In d&s deposds of fibrin, dermat coltagenization and edema were detected EpiderrrIal appendages. rweals presence of Demodex in 12% of patients and in 7 1% of controls. In 40 % of the patients focal necrosis of sweat glands aocompanied for spongioeis of the acmsidngium, hypedrophy. hyperptasia and cellular infiltration were visualred By atiometalograpy technique, traces of mercury were detected in these glands. In co”tr& from El Sagre mercuy was sfso detected I” 40 %. bti without sweat glands necmss Based on these ~~11s and in the occupational exposure of EPF patients (sun and mercury), we suggest that these can play b r& for tngger EPF disease m EL Bagre. .&so the histopathotogicat aiterations of the SMZ, and necrosis of the w.eat glands are umque to this focus.