Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Nov 26;18(12):2532.
doi: 10.3390/ijms18122532.

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Giovanni Paolino  1   2 , Dario Didona  3 , Giuseppe Magliulo  4 , Giannicola Iannella  5 , Biagio Didona  6 , Santo Raffaele Mercuri  7 , Elisa Moliterni  8 , Michele Donati  9 , Andrea Ciofalo  10 , Guido Granata  11 , Patricia Ranuzzi  12 , Vincenzo Falasca  13 , Stefano Calvieri  14
Affiliations
Review

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Giovanni Paolino et al. Int J Mol Sci. .

Abstract

Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed "paraneoplastic pemphigus". We included also papers in French, German, and Spanish. We found 613 papers for "paraneoplastic pemphigus". Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.

Keywords: oncology; oral lesions; paraneoplastic pemphigus; therapy.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Paraneoplastic pemphigus (PNP) oral lesions. Ulceration involve oral mucosal surface and tongue. Stomatitis is also visible.
Figure 2
Figure 2
PNP peri-oral lesions. Ulceration with crusting over the peri-oral region and lips.
Figure 3
Figure 3
PNP skin lesions. Diffuse erythema, exfoliative erythroderma, erosions, or ulcerations on the trunk and abdomen.
Figure 4
Figure 4
PNP head and neck lesions. Erythema and ulceration with oozing and crusting.
Figure 5
Figure 5
Intense, band-like inflammatory infiltrate, with minimal supra-basal acantholysis (H&E, magnification 20×).
Figure 6
Figure 6
Basal cell vacuolar degeneration, dyskeratotic and necrotic keratinocytes, lymphocytic inflammation with lymphocytic exocytosis. (H&E, magnification 30×).
Figure 7
Figure 7
Direct immunofluorescence shows IgG deposition in the epidermal intercellular spaces and in the basement membrane (20×).
See this image and copyright information in PMC

References

    1. Anhalt G.J., Kim S.C., Stanley J.R., Korman N.J., Jabs D.A., Kory M., Izumi H., Ratrie H., Mutasim D., 3rd, Ariss-Abdo L. Paraneoplastic pemphigus. An autoimmune mucocu-taneous disease associated with neoplasia. N. Engl. J. Med. 1990;323:1729–1735. doi: 10.1056/NEJM199012203232503. - DOI - PubMed
    1. Kelly S., Schifter M., Fulcher D.A., Lin M.W. Paraneoplastic pemphigus: Two cases of intra-abdominal malignancy presenting solely as treatment refractory oral ulceration. J. Dermatol. 2015;42:300–304. doi: 10.1111/1346-8138.12753. - DOI - PubMed
    1. Sehgal V.N., Srivastava G. Paraneoplasticpemphigus/paraneoplastic autoimmune multiorgansyn-drome. Int. J. Dermatol. 2009;48:162–169. doi: 10.1111/j.1365-4632.2009.03995.x. - DOI - PubMed
    1. Baum S., Sakka N., Artsi O., Trau H., Barzilai A. Diagnosis and classification of autoimmune blistering diseases. Autoimmun. Rev. 2014;13:482–489. doi: 10.1016/j.autrev.2014.01.047. - DOI - PubMed
    1. Nguyen V.T., Ndoye A., Bassler K.D., Shultz L.D., Shields M.C., Ruben B.S., Webber R.J., Pittelkow M.R., Lynch P.J., Grando S.A. Classification, clinical manifestations, and im-munopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multi-organ syndrome: A reappraisal of paraneoplastic pemphigus. Arch. Dermatol. 2001;137:193–206. - PubMed

MeSH terms

Substances

LinkOut - more resources