Background: Pemphigus vulgaris (PV) is an autoimmune bullous lesion of the skin and mucous membranes characterized by suprabasal clefting and acantholysis. The responsible autoantibody is desmoglein 3, a protein constituent of the desmosome. The diagnosis of PV is based on histological examination and immunofluorescence study. In addition, cytological smears could also be informative for the initial diagnosis of PV. Cases: Fifteen patients, 7 men and 8 women, with skin bullous disease clinically suspected of being PV were selected for cytological study. The bullae were ruptured and two smears were prepared from the fluid and stained with the Wright-Giemsa and Papanicolaou methods. The smears were taken from both new and old bullae. They showed acantholytic cells (Tzanck cells), some with amoeboid-like cytoplasmic projections, eosinophils, basophils, various forms of typical and atypical lymphocytes with irregular nuclear borders and cerebriform nuclei and dysplastic acantholytic cells with a high nucleo-cytoplasmic ratio and prominent nucleoli. The dysplastic cells were seen only in the old bullae. Histological examination and immunofluorescence study of the lesions confirmed the diagnosis of PV. Conclusion: PV can be diagnosed by cytology. Without a clinical history of PV, the presence of atypical lymphocytes and dysplastic cells may lead to a false diagnosis of malignancy.

Keywords:
Skin, Pemphigus vulgaris, Cytological findings, Dysplastic acantholytic cells, Atypical lymphocytes
1.
Graham JH, Bingul O, Burgoon C: Cytodiagnosis of inflammatory dermatoses pitfalls in evaluation of smears. Arch Dermatol 1963;87:158–167.
2.
Scully C, Challacombe SJ: Pemphigus vulgaris: update on etiopathogenesis, oral manifestations and management. Crit Rev Oral Biol Med 2002;13:397–408.
3.
Pavlovsky L, Mimouni D, Halachmi S, Katzenelson V, David M: Pemphigus mimicking skin diseases – atypical presentation delaying correct diagnosis: case series of five patients. Isr Med Assoc J 2008;10:433–434.
4.
Liu Z, Li N, Diaz LA: Immunopathological mechanisms of acantholysis in pemphigus vulgaris: an explanation by ultrastructural observations. J Invest Dermatol DOI: 10.1111/j.0022-202X.2004.22438.x.
5.
Darling MR, Daley T: Blistering mucocutaneous diseases of the oral mucosa – a review. 2. Pemphigus vulgaris. J Can Dent Assoc 2006;72:63–66.
6.
Thorat MS, Raju A, Pradeep AR: Pemphigus vulgaris: effects on periodontal health. J Oral Sci 2010;52:449–454.
7.
Camacho-Alonso F, López-Jornet P, Bermejo-Fenoll A: Pemphigus vulgaris. A presentation of 14 cases and review of the literature. Med Oral Patol Oral Cir Bucal 2005;10:282–288.
8.
Coscia-Porrazzi L, Maiello FM, Ruocco V, Pisani M: Cytodiagnosis of oral pemphigus vulgaris. Acta Cytol 1985;29:746–749.
9.
Cooke BED: Exfoliative cytology in evaluating oral lesions. J Dent Res 1963;2:343–347.
10.
Ohta M, Osawa S, Endo H, Kuyama K, Yamamoto H, Ito T: Pemphigus vulgaris confined to the gingiva: a case report. Int J Dent DOI: 10.1155/2011/207153.
11.
Caltabiano R, Magro G, Puzzo L, Vasquez E, De Pasquale R: Unusual clinical and pathological features in pemphigus vulgaris: a potential diagnostic pitfall. Case Report Med DOI: 10.1155/2011/518758.
12.
Kobayashi TK, Kaneko C, Sugishima S, Kusukawa J, Kameyema T: Scrape cytology of oral pemphigus. Report of a case with immunocytochemistry and light scanning electron and transmission electron microscopy. Acta Cytol 1999;43:289–294.
13.
Gupta S, Sodhani P, Jain S: Acantholytic cells exfoliated from pemphigus vulgaris of the uterine cervix. A case report. Acta Cytol 2003;47:795–797.
14.
McQueen A, Campbell HD: Pemphigus vulgaris: cytodiagnosis of the oral lesion. Br J Oral Surg 1972;10:69–72.
15.
Lobo AZ, Sousa JX Jr, Aoki V, Sotto MN, Aida Gay de Pereyra E, Maruta CW, Santi CG: Pemphigus vulgaris of the uterine cervix: misinterpretation of Papanicolaou smears. Acta Derm Venereol 2006;86:355–356.
16.
Onuma K, Kanbour-Shakir A, Modery J, Kanbour A: Pemphigus vulgaris of the vagina – its cytomorphologic features on liquid-based cytology and pitfalls: case report and cytological differential diagnosis. Diagn Cytopathol 2009;37:832–835.
17.
Kobayashi TK, Ueda M, Nishino T, Kibe S, Higashida T, Watanabe S: Scrape cytology of pemphigus vulgaris of the nipple, a mimicker of Paget’s disease. Diagn Cytopathol 1997;16:156–159.
18.
Balighi K, Moeineddin F, Rajaee AR, Nikoo A: Bullous mycosis fungoides: a case report. Dermatol Online J 2008;14:11.
19.
Bowman PH, Hogan DJ, Sanusi ID: Mycosis fungoides bullosa: report of a case and review of literature. J Am Acad Dermatol 2001;45:934–939.
© 2012 S. Karger AG, Basel
2012
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.