Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil

Barcelos, Vanessa Martins; Vale, Everton Carlos Siviero do; Araujo, Marcelo Grossi; Bittencourt, Flávia Vasques

doi:10.1016/j.abd.2023.03.004

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ORIGINAL ARTICLE • An. Bras. Dermatol. 99 (1) • Jan-Feb 2024 • https://doi.org/10.1016/j.abd.2023.03.004 copy

Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil^☆ ☆ Study conducted at the Service of Dermatology, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Background:

Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject.

Objective:

To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil.

Methods:

An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease.

Results:

A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030).

Study limitations:

The assessed population comes from a tertiary care service that is not a reference for the entire state.

Conclusions:

Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

KEYWORDS
Autoimmunity; Endemic diseases; Pemphigus; Skin diseases, vesiculobullous

	Diagnosis		p-value
	EPF	PV	p-value
Sex
Male	46.9%	34.5%	0.165
Female	53.1%	65.5%
Sunlight exposure
No	79.7%	82.8%	0.665
Yes	20.3%	17.2%
Physical stress
No	95.3%	94.8%	0.902
Yes	4.7%	5.2%
Emotional stress
No	68.7%	69.0%	0.980
Yes	31.3%	31.0%
Medication
No	95.3%	96.6%	0.730
Yes	4.7%	3.4%
Insect bites
No	92.2%	96.6%	0.301
Yes	7.8%	3.4%
Family history of pemphigus
No	85.9%	91.4%	0.346
Yes	14.1%	8.6%
Familial autoimmune disease
No	67.2%	70.7%	0.677
Yes	32.8%	29.3%
Level of schooling
Incomplete Elementary School	45.3%	43.1%
Complete Elementary School	7.8%	15.5%
Incomplete High School	12.5%	10.3%	0.846
Complete High School	29.7%	27.6%
Incomplete Higher Education	3.1%	1.7%
Complete Higher Education	1.6%	1.7%
Residence area ^* * There is statistical difference between the evaluated groups.
Urban	46.9%	87.9%
Rural	32.8%	6.9%	0.000
Periurban	20.3%	5.2%
Forests ^* * There is statistical difference between the evaluated groups.
No	28.1%	60.3%	0.000
Yes	71.9%	39.7%
Rivers/streams ^* * There is statistical difference between the evaluated groups.
No	45.3%	74.1%	0.001
Yes	54.7%	25.9%
Reccurrence ^* * There is statistical difference between the evaluated groups.
No	40.6%	20.7%	0.018
Yes	59.4%	79.3%

	Diagnosis
	EPF (n = 64)		PV (n = 58)
	n	%	n	%
Metropolitan region	38	59.4	41	70.7
Northern MG	9	14.1	4	6.9	χ2 (4.15;4)
Zona da Mata	7	10.8	3	5.2	p-value = 0.386
Vale do Rio Doce	6	9.4	4	6.9
Southern MG	4	6.3	6	10.3

	EPF (n = 64)		PV (n = 58)
	n	%	n	%
Houseworkers	11	17.2	8	13.8
Primary sector	13	20.3	7	12.1	χ2 (13.226;4)
Secondary sector	10	15.6	8	13.8	p-value = 0.010
Tertiary sector	20	31.3	34	58.6
Students	10	15.6	1	1.7

	Median (Q1 – Q3)		p-value
	EPF	PV	p-value
Age (years)	30 (16.3 – 47.8)	45 (33 – 55.3)	0.001
Disease duration (years)	4 (4 – 14.8)	8 (5 – 13.3)	0.949
Systemic medications	2 (2 – 2)	2.5 (2 – 3)	0.002

	Pimentel (2008)	Current study (2021)
Sex
Female	51 (54.3%)	34 (53.1%)	χ2 (0.0196;1)
Male	43 (45.7%)	30 (46.9%)	p-value = 0.889
Age (years)
0 to 12	7 (7.4%)	4 (6.3%)
13 to 21	18 (19.1%)	18 (28.1%)	χ2 (6.74;5)
22 to 45	44 (46.8%)	25 (39.1%)	p-value = 0.241
46 to 65	15 (15.9%)	13 (20.3%)
>65	4 (4.4%)	4 (6.3%)
Unknown	6 (6.4%)	0
Family history of pemphigus
Yes	7 (7.4%)	9 (14.1%)
No	78 (83%)	55 (85.9%)	-
Unknown	9 (9.6%)	0
Occupation
Houseworkers	23 (24.5%)	11 (17.2%)
Primary sector	19 (20.2%)	13 (20.3%)	-
Secondary sector	4 (4.3%)	10 (15.6%)
Tertiary sector	27 (28.7%)	20 (31.3%)
Students	11 (11.7%)	10 (15.6%)
Unknown	10 (10.6%)	0
Residence area ^* * There is statistical difference between the evaluated groups.
Rural/Periurban	40 (42.6%)	34 (53.1%)	χ2 (7.04;2)
Urban	45 (47.9%)	30 (46.9%)	p-value = 0.030
Unknown	9 (9.5%)	0

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[1] ⁎ Corresponding author. E-mail: barcelosmvanessa@gmail.com (V.M. Barcelos).