Clinical Characteristics, Comorbidities, and Treatment in Patients with Pemphigus-A Single-Center Retrospective Study

被引:0
作者
Welc, Natalia [1 ]
Wazniewicz, Sandra [1 ]
Gluszak, Pawel [1 ]
Spalek, Maciej [1 ]
Seraszek-Jaros, Agnieszka [2 ]
Jalowska, Magdalena [1 ]
Dmochowski, Marian [1 ]
机构
[1] Poznan Univ Med Sci, Dept Dermatol, Autoimmune Blistering Dermatoses Sect, PL-61701 Poznan, Poland
[2] Poznan Univ Med Sci, Dept Bioinformat & Computat Biol, PL-61701 Poznan, Poland
关键词
pemphigus; comorbidity; treatment; epidemiology; AUTOIMMUNE BLISTERING DISORDERS; BULLOUS DISEASE; FOLIACEUS; VULGARIS; EPIDEMIOLOGY; MARKER; COMPLICATIONS; POLYMORPHISMS; INVOLVEMENT; VALIDATION;
D O I
10.3390/antib13040103
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Background/Objectives: Pemphigus comprises a diverse group of disorders within the autoimmune bullous dermatoses (AIBDs) spectrum. Among these, pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the most commonly encountered variants. Despite its rarity, this condition can pose a life-threatening risk. We aimed to assess clinical characteristics, comorbidities, medication, as well as the treatment of various types of pemphigus in pemphigus patients. Methods: We gathered data from 69 patients treated in the Department of Dermatology in the years 2016-2023. The investigation included sex, age at diagnosis, type of pemphigus, comorbidities and medications, presence of neoplasms and treatment of pemphigus, as well as enzyme-linked immunosorbent assay (ELISA) and direct immunofluorescence (DIF) results. The data were statistically analyzed with the p-value set at 0.05. Results: The study group comprised 69 patients, including 41 women and 28 men. The mean age at diagnosis was 56.89 years +/- 15.42 years. A total of 79.31% of the patients were diagnosed with PV and the following 26.09% with PF. The most common comorbidities were arterial hypertension, hypercholesterolemia, and diabetes mellitus. The dominant treatment regimen was the systemic use of glucocorticosteroids (GCSs; 90% and 94% of PV and PF patients, respectively). More than half of the patients received at least one GCS-sparing treatment, including dapsone and rituximab. We observed a significantly frequent presence of IgG deposits in DIF in patients with PF (p = 0.0217) and a subsequent correlation between the concurrent presence of IgG deposits in DIF and anti-DSG1 antibodies in ELISA testing (p = 0.0469). The combination of IgG, IgG1, IgG4, and C3 deposits was more often existent in PF patients (p = 0.0054) and the combination of IgG4 and C3 deposits in PV patients (p = 0.0339). We also found a positive correlation between the level of anti-DSG1 antibodies and the age at diagnosis (p = 0.0298). Conclusions: Patients with pemphigus are very often diagnosed with significant comorbidities and take diverse medication, which shows that the treatment of pemphigus should follow a multidisciplinary approach. Accurate analysis of the clinical condition of the patients, as well as the results of the ELISA panel or DIF, is crucial for a successful diagnostic and therapeutic process.
引用
收藏
页数:13
相关论文
共 66 条
[1]   Polymorphisms of HLA microsatellite marker in Tunisian pemphigus foliaceus [J].
Abida, O. ;
Mahfoudh, N. ;
Kammoun, A. ;
Gaddour, L. ;
Hakim, F. ;
Toumi, A. ;
Masmoudi, A. ;
Ben Ayed, M. ;
Turki, H. ;
Masmoudi, H. ;
Makni, H. .
HUMAN IMMUNOLOGY, 2013, 74 (01) :104-109
[2]   Tunisian endemic pemphigus foliaceus is associated with the HLA-DR3 gene: anti-desmoglein 1 antibody-positive healthy subjects bear protective alleles [J].
Abida, O. ;
Zitouni, M. ;
Kallel-Sellami, M. ;
Mahfoudh, N. ;
Kammoun, A. ;
Ben Ayed, M. ;
Masmoudi, A. ;
Mokni, M. ;
Fezzaa, B. ;
Ben Osman, A. ;
Kammoun, M. R. ;
Turki, H. ;
Makni, H. ;
Gilbert, D. ;
Joly, P. ;
Tron, F. ;
Makni, S. ;
Masmoudi, H. .
BRITISH JOURNAL OF DERMATOLOGY, 2009, 161 (03) :522-527
[3]   Serum levels of soluble PD1 in pemphigus vulgaris: A useful marker for disease severity [J].
Abu Zeid, Ola ;
Rashed, Laila ;
Khalifa, Shimaa ;
Saleh, Marwah Adly ;
Ragab, Nanis .
JOURNAL OF COSMETIC DERMATOLOGY, 2021, 20 (04) :1298-1302
[4]   MAJOR HISTOCOMPATIBILITY COMPLEX HAPLOTYPES AND CLASS-II GENES IN NON-JEWISH PATIENTS WITH PEMPHIGUS VULGARIS [J].
AHMED, AR ;
WAGNER, R ;
KHATRI, K ;
NOTANI, G ;
AWDEH, Z ;
ALPER, CA ;
YUNIS, EJ .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1991, 88 (11) :5056-5060
[5]   Epidemiologic Study of 20 Cases of Pemphigus at Hospital Clinico Universitario Virgen de laVictoria de Malaga, Spain [J].
Alcaide-Martin, A. J. ;
Gallardo-Perez, M. A. ;
Castillo-Munoz, R. ;
Mendiola Fernandez, M. V. ;
Herrera-Ceballos, E. .
ACTAS DERMO-SIFILIOGRAFICAS, 2010, 101 (06) :524-533
[6]   Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid [J].
Alpsoy, Erkan ;
Akman-Karakas, Ayse ;
Uzun, Soner .
ARCHIVES OF DERMATOLOGICAL RESEARCH, 2015, 307 (04) :291-298
[7]  
Baican A, 2010, INT J DERMATOL, V49, P768, DOI 10.1111/j.1365-4632.2009.04345.x
[8]   Is conjunctival mucous involvement a marker of severity in pemphigus vulgaris? [J].
Balica, S. ;
Livideanu, C. Bulai ;
Fournie, P. ;
Fortenfant, F. ;
Soler, V. ;
Barbarot, S. ;
Paul, C. .
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY, 2013, 27 (04) :520-521
[9]   Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil [J].
Barcelos, Vanessa Martins ;
do Vale, Everton Carlos Siviero ;
Araujo, Marcelo Grossi ;
Bittencourt, Flavia Vasques .
ANAIS BRASILEIROS DE DERMATOLOGIA, 2024, 99 (01) :43-52
[10]   COMPARATIVE EPIDEMIOLOGY OF PEMPHIGUS IN TUNISIA AND FRANCE - UNUSUAL INCIDENCE OF PEMPHIGUS FOLIACEUS IN YOUNG TUNISIAN WOMEN [J].
BASTUJIGARIN, S ;
SOUISSI, R ;
BLUM, L ;
TURKI, H ;
NOUIRA, R ;
JOMAA, B ;
ZAHAF, A ;
BENOSMAN, A ;
MOKHTAR, I ;
FAZAA, B ;
REVUZ, J ;
ROUJEAU, JC ;
KAMOUN, MR .
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 1995, 104 (02) :302-305