Autoimmune pemphigus: diagnostic challenges, interdisciplinary interaction, and therapeutic errors

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Abstract

Patient safety is a fundamental principle in the provision of medical care. In the Russian Federation, continuous efforts are undertaken to reduce the number of adverse events, including through systematic analysis of medical errors and undesirable incidents. To illustrate these issues, the article presents four clinical cases of patients with various forms of acantholytic pemphigus. Particular attention is given to the young age of the patients and the clinical course of specific forms of acantholytic pemphigus. The paper discusses shortcomings in medical care, including violations of differential diagnostic algorithms, lack of interdisciplinary continuity, and therapeutic strategy failures. It is reported that diagnostic and therapeutic errors contribute to a more severe course of dermatosis and the transformation of one pemphigus form into another.

Timely diagnosis of pemphigus, which belongs to the group of autoimmune bullous dermatoses, is a crucial step in patient management and prevents progression of autoimmune forms in the absence of pathogenetic therapy. Diagnostic criteria include medical history, clinical presentation, cytological examination for acantholytic cells from the base of mucosal and/or skin erosions, histopathological examination of skin biopsy samples from lesions (including direct immunofluorescence methods), and/or indirect immunofluorescence testing of serum to detect IgG and IgA autoantibodies.

The article emphasizes the importance of adherence to clinical guidelines, increasing awareness among physicians of related specialties about current features of bullous dermatoses, and improving coordination among specialists from different disciplines. Addressing these issues will improve the quality of medical care and enhance patient safety.

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About the authors

Marina M. Tlish

Kuban State Medical University

Email: tlish_mm@mail.ru
ORCID iD: 0000-0001-9323-4604
SPIN-code: 8452-4062

MD, Dr. Sci. (Medicine), Professor

Russian Federation, 179 Rashpilevskaya st, Krasnodar, 350051

Taisiya G. Kuznetsova

Kuban State Medical University

Author for correspondence.
Email: taya1504@mail.ru
ORCID iD: 0000-0002-0426-5167
SPIN-code: 1315-2363

MD, Cand. Sci. (Medicine), Assistant Professor

Russian Federation, 179 Rashpilevskaya st, Krasnodar, 350051

Zhanna Yu. Naatyzh

Kuban State Medical University

Email: nzhannau@mail.ru
ORCID iD: 0000-0001-9754-5063
SPIN-code: 3680-1603

MD, Cand. Sci. (Medicine), Assistant Professor

Russian Federation, 179 Rashpilevskaya st, Krasnodar, 350051

Ekaterina A. Inyukhina

Clinical Skin and Venereal Diseases Dispensary

Email: chernenko_ea@mail.ru
ORCID iD: 0000-0003-0919-1184
SPIN-code: 7616-5864
Russian Federation, Krasnodar

Anastasia G. Usikova

Clinical Skin and Venereal Diseases Dispensary

Email: usikova1992@mail.ru
ORCID iD: 0000-0003-1547-931X
SPIN-code: 6185-1682
Russian Federation, Krasnodar

References

  1. Tlish MM, Kuznetsova TG, Naatyzh ZhYu, Sycheva NL. Differential diagnostic search in dermatologic practice and its effect on quality of medical care: Clinical cases. Kuban Scientific Medical Bulletin. 2023;30(6):89–101. doi: 10.25207/1608-6228-2023-30-6-89-101 EDN: LATEBF
  2. Tlish MM, Kuznetsova TG, Naatyzh ZhYu. Clinical analysis of iatrogenic diseases in the provision of medical care to patients with psoriasis. Vrach. 2018;29(11):82–88. doi: 10.29296/25877305-2018-11-19 EDN: YOUBZB
  3. Tlish MM, Kuznetsova TG, Naatyzh ZhYu, et al. Clinical guidelines determine the quality of medical care in dermatovenerology. Russian Medical Inquiry. 2020;4(10):637–641. doi: 10.32364/2587-6821-2020-4-10-637-641 EDN: EXBKQX
  4. Teplyuk NP, Kolesova YV, Mak DV, et al. Pemphigus: New approaches to diagnosis and disease severity assessment. Russian Journal of Skin and Venereal Diseases. 2023;26(5):515–526. doi: 10.17816/dv492306 EDN: MSAHYT
  5. Timóteo RP, Pessoa-Gonçalves YM, do Carmo Neto JR, et al. A global view of pemphigus: Geographical variations. Clin Rev Allergy Immunol. 2024;66(1):14–29. doi: 10.1007/s12016-024-08980-w
  6. Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: Unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. 1995;104(2):302–305. doi: 10.1111/1523-1747.ep12612836
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Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. Patient B., 31 years old. Clinical manifestations of pemphigus vegetans on the skin of the abdomen at the time of admission.

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3. Fig. 2. Patient B., 31 years old, pemphigus vegetans. Lesion of the oral mucosa (a, b) at the time of admission.

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4. Fig. 3. Patient B., 31 years old. Dynamics of the skin pathological process at the end of inpatient treatment (30th day): clinical manifestations regressed (a, b).

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5. Fig. 4. Patient G., 73 years old, pemphigus foliaceus. Widespread erythematous-squamous rash on the skin of the trunk and upper limbs (a, b), on the skin of the thighs (с) at the time of admission.

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6. Fig. 5. Patient S., 62 years old, pemphigus vulgaris. Erosive lesions in the folds under the mammary glands (a), on the skin abdomen (b) and back (с) in the progression phase against the background of glucocorticosteroid therapy.

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7. Fig. 6. Patient S., 32 years old. Clinical manifestations of pemphigus vulgaris on the skin of the back and shoulder (a, b), on the skin of the face with transformation of seborrheic pemphigus into vulgaris (с) against the background of drug therapy with systemic glucocorticoids.

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