Atypical pemphigus
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1
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, U.O. Dermatologia, Italy
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2
Università di Milano - Bicocca, Italy
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3
Università degli Studi di Milano, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Italy
We diagnosed 300 cases of pemphigus vulgaris (PV) and foliaceus (PF), collecting clinical data, skin and blood serum. Methods used were: direct immunofluorescence (DIF); indirect IF (IIF) on normal human skin (NHS); ELISA for Desmoglein (DSG) 1-3 and bullous pemphigoid (BP) antigens 180-230; transfected cells for DSG1-3. Atypical clinical presentations were observed in case of herpetiform pemphigus (HP). HP demonstrated IgA intercellular (IC) deposits, anti DSG3 or IC staining in absence of DSG1-3 reactivity (Desmocollin+). Concurrent neoplasia were detected in 2 HP patients. Localized oral pemphigus vulgaris (OPV) were also observed, IgA and IgG mucosal deposits were demonstrated in 2 cases (1 case associated with thyroid neoplasia). In 2 exceptional cases OPV and PF firstly occurred during pregnancy. Patients affected by chronic lymphatic leukemia showed aggressive paraneoplastic pemphigus or a common OPV. In children PV, PF and a case of IgA pemphigus were observed. Typical cases of subcorneal pustolosis like IgA pemphigus were also observed in adults. Looking at serology anti DSG1-3 and anti BP180-230 were simultaneously detected by ELISA in some patients (aggressive cases?). Complete clinical remission was observed in patients with persistent high titre of antibodies. IIF on NHS stained lower part of epidermis (DSG3+) or upper part of epidermis (DSG1+). The specificity of cells transfected by DSG1-3 and BP230 was also confirmed in all cases analyzed. In conclusion the majority of atypical pemphigus cases should be identify and classify by old and new technologies, while antibody titre by ELISA not always was in correlation with clinical evolution.
Keywords:
Pemphigus,
desmoglein,
Desmocollin,
Direct immunofluorescence,
ELISA
Conference:
15th International Congress of Immunology (ICI), Milan, Italy, 22 Aug - 27 Aug, 2013.
Presentation Type:
Abstract
Topic:
Adaptive Immunity
Citation:
Berti
E,
Venegoni
L,
Fanoni
D,
Lunardon
L and
Muratori
S
(2013). Atypical pemphigus.
Front. Immunol.
Conference Abstract:
15th International Congress of Immunology (ICI).
doi: 10.3389/conf.fimmu.2013.02.00804
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Received:
19 Jun 2013;
Published Online:
22 Aug 2013.
*
Correspondence:
Prof. Emilio Berti, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, U.O. Dermatologia, Milan, Italy, emilio.berti@unimib.it