Pemphigus: New approaches to diagnosis and disease severity assessment

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Abstract

Pemphigus represents a group of severe and potentially fatal autoimmune bullous diseases. Pemphigus clinical findings represents skin and/or mucous membranes keratinocytes acantholysis with intraepithelial blisters and erosions formation which is life-threatening and, if relapsed, significantly reduce the patients’ quality of life. The on-time disease diagnosis is a key to successful pemphigus management and timely treatment onset allowing intensive disease progression avoidance.

The article reviews up-to-date diagnosing methods and assessment measure for monitoring pemphigus severity. In addition to standard diagnosis methods, such as clinical findings, histological examination, direct and indirect immunofluorescence, and enzyme immunoassay (ELISA), the article focuses on revieing new diagnostic and disease severity prediction approaches, such as microRNAs expression and skin and mucous membranes microbiome analysis since false positive and false negative results in histological and immunohistochemical analysis as well as the laboratories insufficient equipment makes finding pemphigus new diagnostic and prognostic methods a relevant challenge.

About the authors

Natalia P. Teplyuk

The First Sechenov Moscow State Medical University (Sechenov University)

Email: teplyukn@gmail.com
ORCID iD: 0000-0002-5800-4800
SPIN-code: 8013-3256

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Yulia V. Kolesova

The First Sechenov Moscow State Medical University (Sechenov University)

Email: jusamilutin@rambler.ru
ORCID iD: 0000-0002-3617-2555
SPIN-code: 1441-8730

Graduate Student, Assistant Lecturer

Russian Federation, Moscow

Daria V. Mak

The First Sechenov Moscow State Medical University (Sechenov University)

Email: bedariaaa@gmail.com
ORCID iD: 0000-0002-7020-0572
SPIN-code: 8204-4555

Graduate Student

Russian Federation, Moscow

Anfisa A. Lepekhova

The First Sechenov Moscow State Medical University (Sechenov University)

Email: anfisa.lepehova@yandex.ru
ORCID iD: 0000-0002-4365-3090
SPIN-code: 3261-3520

MD, Cand. Sci. (Med.), Associate Professor

Russian Federation, Moscow

Stepan V. Toshchakov

National Research Centre "Kurchatov Institute"

Author for correspondence.
Email: stepan.toshchakov@gmail.com
ORCID iD: 0000-0001-7549-3450
SPIN-code: 8994-5224

National Research Centre "Kurchatov Institute"

Russian Federation

Tatiana A. Fedotcheva

The Russian National Research Medical University named after N.I. Pirogov

Email: tfedotcheva@mail.ru
ORCID iD: 0000-0003-4998-9991
SPIN-code: 1261-5650

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

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Supplementary files

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2. Fig. 1. Modern methods of diagnosis of autoimmune pemphigus (a straight line is already used diagnostic methods, a dotted line is promising methods). АП ― autoimmune pemphigus; ВП ― vulgar pemphigus; ЛП ― leaf-shaped pemphigus; ИФА (ELISA) ― enzyme immunoassay; РИФ ― immunofluorescence reaction.

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3. Fig. 2. Clinical picture of vulgar pemphigus on the skin: erosions with fragments of epidermis tires on the periphery are noted.

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4. Fig. 3. Clinical picture of leaf-shaped pemphigus: erosions covered with thin, layered scales-crusts.

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5. Fig. 4. Histological picture of vulgar pemphigus: acantholysis at the level of the spiny layer, which led to the formation of an intraepidermal bubble.

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6. Fig. 5. Histological picture of leaf-shaped pemphigus (3 months from the onset of the disease): formation of intraepidermal microabsesses.

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7. Fig. 6. Direct immunofluorescence reaction in a patient with vulgar pemphigus: fixation of IgG in the spiny layer of the epidermis.

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