Difficulties in diagnosing pemphigus vulgaris

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Abstract

Vulgar pemphigus is a rare, potentially fatal autoimmune disease characterized by formation of intraepidermal blisters and extensive erosions on the skin and mucous membranes. Despite significant advances in investigation of the pathogenesis and treatment of pemphigus vulgaris, the diagnosis of this disease remains challenging. The difficulty in early diagnosis of bullous dermatoses, particularly pemphigus vulgaris, is due to the non-specific nature of the initial clinical manifestations. The typical primary lesion of the oral mucosa (erosions, aphtha-like elements) often leads to misinterpretation of the symptoms and delayed diagnosis. The problem is exacerbated by the lack of awareness among primary care physicians (dentists, otolaryngologists, and general practitioners) about this condition. The paper focuses on the difficulties in diagnosing pemphigus vulgaris. A case narrative described a patient whose disease began with nonspecific rash on the oral mucosa which made it difficult to make an accurate diagnosis for a long time. Based on the results of a cytological examination of smears from the surface of new erosions, the oncologist diagnosed “N2NxMx cancer of the lower lip” and performed radiation therapy, which aggravated the disease. The paper discusses possible causes of diagnostic errors.

About the authors

Margarita R. Rakhmatulina

State Research Center of Dermatovenereology and Cosmetology

Email: rahmatulina@cnikvi.ru
ORCID iD: 0000-0003-3039-7769
SPIN-code: 6222-8684

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

Russian Federation, Moscow

Egana S. Mehdieva

State Research Center of Dermatovenereology and Cosmetology

Email: dr.mekhdieva@mail.ru
ORCID iD: 0009-0004-8286-9427

MD, Dermatovenereologist

Russian Federation, Moscow

Viktoria L. Te

Russian Medical Academy of Continuous Professional Education

Author for correspondence.
Email: vika-pak_123@bk.ru
ORCID iD: 0009-0009-8506-8162
SPIN-code: 2423-2889
Russian Federation, 2/1 bldg 1 Barrikadnaya street, 125993 Moscow

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Supplementary files

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1. JATS XML
2. Fig. 1. Swollen lower lip covered with thick purulent discharge

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3. Fig. 2. Tightly adhering serous-hemorrhagic crusts on the scalp skin: a — occipital region; б — parietal region; в — frontotemporal area

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4. Fig. 3. Bright pink wet erosions covered with multilayered dense hemorrhagic crusts

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5. Fig. 4. Multiple bright pink wet erosions covered with massive crusts

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6. Fig. 5. Extensive wet erosions on the skin of the lumbar and intergluteal areas

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7. Fig. 6. Histological examination of a skin biopsy (hematoxylin and eosin staining, magnification × 100)

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8. Fig. 7. Immunofluorescence testing with anti-IgG, IgA, and IgM-antibodies in a biopsy of apparently intact skin: weak and moderate fixation of IgG in the intercellular spaces of all epidermis layers of th (“grid”-like)

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Copyright (c) 2025 Rakhmatulina M.R., Mehdieva E.S., Te V.L.

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