Data on HLA class I/II profile in Brazilian pemphigus patients.

Pemphigus are blistering autoimmune diseases related with genetic and environmental factors. Here we describe HLA genotyping in pemphigus patients. First, we review the HLA class I/II data on pemphigus reported in Brazilian samples and then present the HLA class I (-A, -B, -C) and class II (-DRB1, -DQA1, -DQB1) alleles related to susceptibility/resistance to pemphigus by comparing 86 patients with pemphigus foliaceus, 83 patients with pemphigus vulgaris, and 1592 controls from the northeastern region of the state of São Paulo, Southeastern Brazil. The data presented here are related to the manuscript "Differential HLA class I and class II associations in Pemphigus Foliaceus and Pemphigus Vulgaris patients from a prevalent Southeastern Brazilian region" Brochado et al. (2016) [1].

Specifications Table

Value of the data

The literature review regarding HLA class I/II data on pemphigus is shown in tables comparing different studied Brazilian populations.

The northeastern region of the state of São Paulo, Southeastern Brazil, is prevalent for both clinical forms of pemphigus–pemphigus foliaceus and pemphigus vulgaris, enabling a comparative study.

HLA class I/II frequencies are detailed comparing pemphigus foliaceus and pemphigus vulgaris patients from the same endemic region.

Data

Table 1, Table 2 describe the HLA class I and II data related to susceptibility/resistance to pemphigus foliaceus and pemphigus vulgaris in reviewed Brazilian reports. Table 3, Table 4, Table 5, Table 6, Table 7, Table 8 show the HLA class I (-A, -B, -C) and class II (-DRB1, -DQA1, -DQB1) profile performed in pemphigus foliaceus and pemphigus vulgaris patients from Southeastern Brazil.

Table 1

Brazilian reports on pemphigus foliaceus associated alleles.

References		Brazilian population	Patients/Controls	DRB1*	P-value, RR or OR	DQA1*	P-value, RR	DQB1*	P-value, RR or OR
Susceptibility – Pemphigus Foliaceus
PetzL-Erler et al. (1989) [2]	North to Southwest of the state of Paraná		48/74	01	Pc=3.3×10−3, RR=6.4
				04	P=3.3×10−3, RR=3.3
Moraes et al. (1991) [3]	State of São Paulo and Brasília city (Federal District)		38/50	01:02	Pc=0.002, RR=7.3
Cerna et al. (1993) [4]	Xavante Indians – Central Brazil		10/74	04:04	Pc=0.03, RR=9.6
Moraes et al. (1997) [5]	Terena Indians – state of Mato Grosso do Sul		20/66	04:04	Pc=0.022, OR=6.1			03:02	Pc=0.04, OR=5.2
Pavoni et al. (2003) [6]	State of Mato Grosso do Sul and Paraná		128/402	01	P<10−6, OR=7.4
				01:01	P=0.042, OR=1.83
				01:02	P<10−6, OR=10.36
				01:03	P=0.025, OR=5.41
				04	P<10−6, OR=2.66
				04:04	P=4×10−6, OR=4.58
				04:06	P=5.2×10−6, OR=35.85
				04:10	P=0.046, OR=9.62
				14:06	P=0.04, OR=4.04
				16:01	P=0.017, OR=2.87
Brochado et al. (2016) [1]	Northeastern region of the state of São Paulo		86/1592	01:01	Pc=0.0001, RR=2.18	01	Pc=0.02, RR=1.41	05:01	Pc=2.5×10−10, RR=2.95
				01:02	Pc=5.4e−10, RR=6.06	01:02	Pc=3.6×10−3, RR=2.3
						03	Pc=0.01, RR=1.78
Protection – Pemphigus Foliaceus
Petzl-Erler et al. (1989) [2]	North/Southwest of the state of Paraná		48/74	07	Pc=9×10−3, RR=0.06			02:01	P=8.1×10−3, RR=0.27
Moraes et al. (1991) [3]	State of São Paulo and Brasília city (Federal District)		38/50					02:01	P=0.006, RR=0.04
								06:02	Pc=0.042, OR=0.15
Pavoni et al. (2003) [6]	States of Mato Grosso do Sul and Paraná		128/402	03:01	P=8.7×10−4, OR=0.23
				07:01	P<10−6, OR=0.09
				08	P=1×10−3, OR=0.27
				08:01	P=7.2×10−3, OR=0.07
				11	P<10−6, OR=0.09
				11:01	P<10−6, OR=0.05
				11:04	P=0.03, OR=0.15
				14:02	P=0.018, OR=0.09
				15	P=0.019, OR=0.51
Brochado et al. (2016) [1]	Northeastern region of the state of São Paulo			11:01	Pc=0.027, RR=0.07	02:01	Pc=0.03, RR=0.34	03:01	Pc=0.0002; RR=0.39
				13:01	Pc=0.027, OR=0.20	05	Pc=1.08×10−5, RR=0.28	06:03	Pc=0.023, RR=0.2

RR=Relative risk, OR=odds ratio, Pc=P-values were corrected by the number of alleles tested for each locus.

Table 2

Brazilian reports on pemphigus vulgaris associated alleles.

References	Brazilian population	Patients/Controls	DRB1*	P-value, OR or RR	DQA1*	P-value, OR	DQB1*	P-value, RR
Susceptibility – Pemphigus Vulgaris
Weber et al. (2011) [7]	Southeastern region of the state of São Paulo	36/162	04:02	OR=44.6
			08:04	OR=18.6
			14	OR=4.8
Brochado et al. (2016) [1]	Northeastern region of the state of São Paulo	82/1592	04:02	Pc=5.4×10−10, RR=12.54	03	Pc=0.01, OR=2.04	03:02	Pc=2.5×10−10, RR= 2.95
			08:04	Pc=5.4×10−5, RR=6	03:01	Pc=3.6×10−4, OR=4	05:03	Pc=0.02, OR=2.74
			14:01	Pc=5.4×10−10, RR=7
			14:04	Pc=5.4×10−4, RR=16.64
Protection – Pemphigus Vulgaris
Brochado et al. (2016) [1]	Northeastern region of the state of São Paulo	82/1592	07:01	Pc=0.027, RR=0.28			06:02	Pc=0.0075, RR=0.19

RR=Relative risk, OR=odds ratio, Pc=P-values were corrected by the number of alleles tested for each locus.

Table 3

Allelic HLA-A frequencies among Brazilian pemphigus foliaceus and pemphigus vulgaris patients as compared to controls.

HLA-A*	Controls (n=1592)	Pemphigus foliaceus (n=83)	Pemphigus vulgaris (n=83)
	n (%)	n (%)	n (%)
01	291 (9.14)	12 (7.23)	21 (12.65)
02	1028 (32.29)	31 (18.67)a	38 (22.89)
03	277 (8.70)	18 (10.84)	9 (5.42)
04	1 (0.03)	0	0
06	2 (0.06)	0	0
11	126 (3.96)	16 (9.64)b	11 (6.63)
23	127 (3.99)	8 (4.82)	4 (2.41)
24	324 (10.18)	16 (9.64)	18 (10.84)
25	38 (1.19)	2 (1.20)	4 (2.41)
26	86 (2.70)	5 (3.01)	13 (7.83)c
29	114 (3.58)	4 (2.41)	2 (1.20)
30	163 (5.12)	14 (8.43)	11 (6.63)
31	149 (4.68)	6 (3.61)	4 (2.41)
32	95 (2.98)	3 (1.81)	5 (3.01)
33	73 (2.29)	11 (6.63)d	6 (3.61)
34	18 (0.57)	3 (1.81)	1 (0.60)
36	13 (0.41)	1 (0.60)	1 (0.60)
66	17 (0.53)	3 (1.81)	2 (1.20)
68	205 (6.44)	8 (4.82)	13 (7.83)
69	1 (0.03)	0	0
74	34 (1.07)	5 (3.01)	1 (0.60)
80	2 (0.06)	0	2 (1.20)

RR=Relative Risk, CI=confidence interval.

P=2.10−4, RR=0.57, 95% CI=0.42–0.80.

P=0.04, RR=2.43, 95% CI=1.5–4.0.

P=0.02, RR=2.89, 95% CI=1.65–5.08.

P=0.04, RR=2.89, 95% CI=1.56–5.34.

Table 4

Allelic HLA-B* frequencies among Brazilian pemphigus foliaceus and pemphigus vulgaris patients as compared to controls.

HLA-B*	Controls (n=1592)	Pemphigus foliaceus (n=83)	Pemphigus vulgaris (n=82)
	n (%)	n (%)	n (%)
07	229 (7.19)	13 (7.83)	5 (3.05)
08	111 (3.49)	8 (4.82)	2 (1.22)
13	35 (1.10)	3 (1.81)	4 (2.44)
14	161 (5.06)	23 (13.86)a	4 (2.44)
15	379 (11.90)	12 (7.23)	5 (3.05)b
18	185 (5.81)	3 (1.81)	5 (3.05)
27	45 (1.41)	3 (1.81)	4 (2.44)
35	444 (13.94)	19 (11.45)	26 (15.85)
37	25 (0.79)	3 (1.81)	2 (1.22)
38	60 (1.88)	2 (1.20)	12 (7.32)c
39	113 (3.55)	11 (6.63)	9 (5.49)
40	124 (3.89)	10 (6.02)	8 (4.88)
41	31 (0.97)	1 (0.60)	1 (0.61)
42	33 (1.04)	2 (1.20)	3 (1.83)
44	333 (10.46)	14 (8.43)	23 (14.02)
45	45 (1.41)	4 (2.41)	3 (1.83)
47	5 (0.16)	0	0
48	21 (0.66)	3 (1.81)	0
49	68 (2.14)	1 (0.60)	4 (2.44)
50	75 (2.36)	4 (2.41)	4 (2.44)
51	330 (10.36)	8 (4.82)	13 (7.93)
52	49 (1.54)	3 (1.81)	2 (1.22)
53	55 (1.73)	3 (1.81)	8 (4.88)
55	33 (1.04)	2 (1.20)	4 (2.44)
56	4 (0.13)	1 (0.60)	0
57	92 (2.89)	6 (3.61)	10 (6.10)
58	86 (2.70)	4 (2.41)	1 (0.61)
73	4 (0.13)	0	1 (0.61)
81	8 (0.25)	0	1 (0.61)
82	1 (0.03)	0	0

RR=Relative Risk, CI=confidence interval.

P=6×10−4, RR=2.74, 95% CI=1.82–4.12.

P=0.003, RR=0.26, 95% CI=0.10–0.61.

P=0.003, RR=3.88, 95% CI=2.13–7.07.

Table 5

Allelic HLA-C frequencies among Brazilian pemphigus foliaceus and pemphigus vulgaris patients as compared to controls.

HLA-C*	Controls (n=1305)	Pemphigus foliaceus (n=83)	Pemphigus vulgaris (n=82)
	n (%)	n (%)	n (%)
01	55 (2.11)	3 (1.81)	5 (3.05)
02	176 (6.74)	15 (9.04)	10 (6.10)
03	278 (10.65)	14 (8.43)	11 (6.71)
04	475 (18.20)	27 (16.27)	32 (19.51)
05	144 (5.52)	8 (4.82)	13 (7.93)
06	212 (8.12)	14 (8.43)	14 (8.54)
07	578 (22.15)	33 (19.88)	24 (14.63)
08	135 (5.17)	17 (10.24)	5 (3.05)
12	155 (5.94)	10 (6.02)	21 (12.80)a
14	79 (3.03)	0	4 (2.44)
15	111 (4.25)	13 (7.83)	11 (6.71)
16	144 (5.52)	7 (4.22)	10 (6.10)
17	52 (1.99)	3 (1.81)	4 (2.44)
18	16 (0.61)	2 (1.20)	0

RR=Relative Risk, CI=confidence interval

P=0.01, RR=2.16, 95% CI=1.40–3.30.

Table 6

Allelic HLA-DRB1 frequencies among Brazilian pemphigus foliaceus and pemphigus vulgaris patients as compared to controls.

HLA-DRB1⁎	Controls (n=1592)	Pemphigus foliaceus (n=86)	Pemphigus vulgaris (n=82)
	n (%)	n (%)	n (%)
01:01	150 (4.7)	23 (13.4)a	6 (3.7)
01:02	113 (3.6)	37 (21.5)b	3 (1.8)
01:03	22 (0.7)	1 (0.6)	0
03:01	247 (7.8)	10 (5.8)	4 (2.4)
03:02	25 (0.8)	3 (1.7)	2 (1.2)
04	0	2 (1.2)	0
04:01	79 (2.5)	3 (1.7)	2 (1.2)
04:02	65 (2.0)	4 (2.3)	42 (25.6)c
04:03	38 (1.2)	2 (1.2)	4 (2.4)
04:04	88 (2.8)	10 (5.8)	5 (3.1)
04:05	63 (2.0)	4 (2.3)	1 (0.6)
04:06	8 (0.3)	1 (0.6)	0
04:07	27 (0.9)	1 (0.6)	0
04:08	21 (0.7)	2 (1.2)	0
04:10	2 (0.1)	0	0
04:11	54 (1.7)	7 (4.1)	2 (1.2)
07:01	342 (10.7)	6 (3.5)	5 (3.1)d
08:01	71 (2.2)	0	2 (1.2)
08:02	34 (1.1)	3 (1.7)	1 (0.6)
08:03	10 (0.3)	0	0
08:04	42 (1.3)	2 (1.2)	13 (7.9)e
08:07	25 (0.8)	1 (0.6)	0
09:01	41 (1.3)	4 (2.3)	0
10:01	43 (1.4)	1 (0.6)	1 (0.6)
11	0	0	1 (0.6)
11:01	258 (8.1)	1 (0.6)f	9 (5.5)
11:02	72 (2.3)	1 (0.6)	5 (3.1)
11:03	31 (1.0)	0	0
11:04	138 (4.3)	1 (0.6)	4 (2.4)
11:06	1 (0.03)	0	0
11:13	1 (0.03)	0	0
11:18	1 (0.03)	0	0
12:01	35 (1.1)	3 (1.7)	1 (0.6)
12:02	3 (0.1)	0	0
13:01	274 (8.6)	3 (1.7)g	4 (2.4)
13:02	158 (5.0)	2 (1.2)	3 (1.8)
13:03	55 (1.7)	0	2 (1.2)
13:05	2 (0.1)	0	0
13:06	1 (0.03)	0	0
13:21	1 (0.03)	0	0
13:23	1 (0.03)	0	0
13:31	1 (0.03)	0	0
14:01	70 (2.2)	0	25 (15.2)h
14:02	35 (1.1)	1 (0.6)	3 (1.8)
14:04	7 (0.2)	2 (1.2)	6 (3.7)i
14:06	7 (0.2)	1 (0.6)	0
14:09	1 (0.03)	0	0
15:01	194 (6.1)	12 (7.0)	2 (1.2)
15:02	24 (0.8)	0	2 (1.2)
15:03	79 (2.5)	5 (2.9)	0
15:04	1 (0.03)	0	0
15:11	1 (0.03)	0	0
16:01	66 (2.1)	10 (5.8)	2 (1.2)
16:02	56 (1.8)	3 (1.7)	2 (1.2)

RR=relative risk, CI=confidence interval.

P=1×10−4, RR=2.83, 95% CI=1.88–4.28.

P=5×10−10, RR=6.06, 95% CI=4.32–8.49.

P=5.4×10−10, RR=12.54, 95% CI=8.79–17.88.

P=0.027, RR=0.28, 95% CI=0.12–0.67.

P=5.4×10−5, RR=6.0, 95% CI=3.29–10.97.

P=0.027, RR=0.07, 95% CI=0.01–0.50.

P=0.027, RR=0.20, 95% CI=0.06–0.62.

P=5.4×10−10, RR=7.21, 95% CI=4.72–10.99.

P=5.4×10−4, RR=16.64, 95% CI=5.65–48.95.

Table 7

Allelic HLA-DQA1 frequencies among Brazilian pemphigus foliaceus and pemphigus vulgaris patients as compared to controls.

HLA-DQA1⁎	Controls (n=1312)	Pemphigus foliaceus (n=86)	Pemphigus vulgaris (n=82)
	n (%)	n (%)	n (%)
01	760 (29.0)	70 (40.7)a	47 (28.7)
01:02	155 (5.9)	24 (14.0)b	8 (4.9)
01:03	130 (5.0)	3 (1.7)	4 (2.4)
01:06	3 (0.11)	0	0
01:07	4 (0.15)	2 (1.2)	0
01:09	1 (0.04)	0	0
02:01	267 (10.2)	6 (3.5)c	6 (3.7)
03	318 (12.1)	37 (21.5)d	36 (22.0)e
03:01	88 (3.4)	5 (2.9)	20 (12.2)f
04	1 (0.04)	0	0
04:01	146 (5.6)	7 (4.1)	10 (6.1)
04:03	2 (0.1)	0	0
04:04	2 (0.1)	0	0
05	499 (19.0)	9 (5.2)g	27 (16.5)
05:01	201 (7.7)	9 (5.2)	5 (3.1)
05:02	2 (0.1)	0	0
05:10	33 (1.3)	0	1 (0.6)
06:01	12 (0.5)	0	0

RR=relative risk, CI=confidence interval.

P=0.02, RR=1.41, 95% CI=1.16–1.69.

P=3.6×10−3, RR=2.36, 95% CI=1.58–3.52.

P=0.03, RR=0.34, 95% CI=0.15–0.75.

P=0.01, RR=1.78, 95% CI=1.31–2.40.

P=0.01, RR=1.81, 95% CI=1.33–2.46.

P=4×10−4, RR=3.64, 95% CI=2.29–5.75.

P=1.08×10−5, RR=0.28, 95% CI=0.14–0.52.

Table 8

Allelic HLA-DQB1 frequencies among Brazilian pemphigus foliaceus and pemphigus vulgaris patients as compared to controls.

HLA-DQB1*	Controls (n=1411)	Pemphigus foliaceus (n=86)	Pemphigus vulgaris (n=82)
	n (%)	n (%)	n (%)
02:01	233 (8.26)	9 (5.23)	4 (2.44)
02:02	260 (9.21)	6 (3.49)	5 (3.05)
03	2 (0.07)	0	1 (0.61)
03:01	626 (22.18)	15 (8.72)a	30 (18.29)
03:02	297 (10.52)	27 (15.70)	51 (31.10)b
03:03	75 (2.66)	4 (2.33)	1 (0.61)
03:04	3 (0.11)	0	0
03:05	1 (0.04)	0	1 (0.61)
03:19	13 (0.46)	0	2 (1.22)
04	0	1 (0.58)	1 (0.61)
04:01	3 (0.11)	0	0
04:02	170 (6.02)	12 (6.98)	9 (5.49)
05	0	7 (4.07)	22 (13.41)
05:01	328 (11.62)	59 (34.30)c	10 (6.10)
05:02	91 (3.22)	11 (6.40)	3 (1.83)
05:03	69 (2.45)	1 (0.58)	11 (6.71)d
05:05	1 (0.04)	0	0
06	8 (0.28)	0	0
06:01	19 (0.67)	0	3 (1.83)
06:02	263 (9.32)	16 (9.30)	3 (1.83)e
06:03	226 (8.01)	3 (1.74)f	4 (2.44)
06:04	99 (3.51)	1 (0.58)	3 (1.83)
06:09	29 (1.03)	0	0
06:11	5 (0.18)	0	0
16:02	1 (0.04)	0	0

RR=relative risk, CI=confidence interval.

P=2×10−4, RR=0.39, 95% CI=0.24–0.64.

P=2.5×10−10, RR=2.95, 95% CI=2.3–3.80.

P=2.5×10−10, RR=2.95, 95% CI=2.34–3.71.

P=0.02, RR=2.74, 95% CI=1.49–5.08.

P=7.5×10−3, RR=0.19, 95% CI=0.06–0.60.

P=0.02, RR=0.22, 95% CI=0.07–0.67.

Experimental design, materials, and methods

A summary of Brazilian data regarding associations between HLA and pemphigus was obtained in PubMed. A hundred and sixty-nine patients followed up at the University Hospital of the Ribeirão Preto Medical School of the University of São Paulo, Brazil, were evaluated. Eighty-six and 83 patients exhibited PF and PV, respectively. The control group consisted of 1592 healthy individuals living in the northeastern region of the state of São Paulo, Southeastern Brazil. HLA class I and II typing was performed at low/high resolution by using commercial kits, according to the manufacturer׳s protocol (One Lambda Inc., Canoga Park, CA). The allelic frequencies of the HLA class I and II genes were estimated by direct counting. Comparison of allele frequency among the groups was performed by using Fisher׳s exact test or the Chi-square test. Significant P-values were corrected by the number of alleles tested for each locus. The relative risk (RR) 95% was estimated. Statistical analysis was performed with SAS 9.3 (SAS Institute Inc, EUA) and Epi InfoTM 7.0 (CDC, USA) software. Values P≤0.05 were considered significant. All the participants provided an informed written consent to participate in this study. The local Ethics Committee (#12248/2010) approved this study.

Subject area	Biology
More specific subject area	Dermatology, Immunology, and Genetics
Type of data	Tables
How data was acquired	The reviewing of the literature was made by using the Pubmed, and the HLA typing by using PCR-SSOP method
Data format	Analyzed
Experimental factors	DNA blood samples from pemphigus patients and controls
Experimental features	HLA class I and II typing was performed using commercial kits (One Lambda Inc., Canoga Park, CA)
Data source location	Northeastern region of the state of São Paulo, Southeastern Brazil
Data accessibility	Data is with this article