Factors associated with non-pathogenic antibodies against desmoglein-3 in pemphigus foliaceus<sup/>

Vernal, Sebastian; Julio, Tamiris Amanda; Alves, Fernando Henrique; Turatti, Aline; Donadi, Eduardo Antonio; Roselino, Ana Maria

doi:10.1016/j.abd.2023.11.006

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Original Article • An. Bras. Dermatol. 99 (5) • Sep-Oct 2024 • https://doi.org/10.1016/j.abd.2023.11.006 linkcopy

Factors associated with non-pathogenic antibodies against desmoglein-3 in pemphigus foliaceus^☆

Authorship SCIMAGO INSTITUTIONS RANKINGS

Background Anti-desmoglein (Dsg)1 is produced in pemphigus foliaceus (PF), affecting exclusively the skin. Pemphigus vulgaris (PV) shows the production of anti-Dsg3 in the mucosal form, and anti-Dsg1 and 3 in the mucocutaneous form. Anti-Dsg3 autoantibodies have been rarely reported in PF.

Objectives To determine the factors associated with the production and pathogenicity of anti-Dsg3 in PF.

Methods Comparative analytical study of three patients groups: 16 PF-anti-Dsg3+, and 42 PF-anti-Dsg3(-) and 22 PV treatment-naïve cases. Serum was used in the anti-Dsg1 and 3 ELISA, and in immunoblotting (IB) with human epidermis extract. The expression of Dsg1 and 3 in paraffin sections was analyzed by immunohistochemistry (IHC). HLA-DRB1 alleles were compiled from a database.

Results In the PF-anti-Dsg3+ group: age range similar to that of the PV group (p > 0.9999); predominance of the generalized form of PF (p = 0.002); anti-Dsg3 titers lower than those of PV (p < 0.0001); IB confirmed Dsg3 identification in one (8.33%) of 12 patients; IHC showed exclusive cytoplasmic internalization of Dsg1; HLA-DRB1 alleles of susceptibility to PF, with the absence of alleles associated with PV, in the five typed patients.

Study limitations Most of the patients in the PF-anti-Dsg3+ group were undergoing treatment.

Conclusion The presence of anti-Dsg3 antibodies in PF was related to older age (comparable to that of PV) and the generalized form of PF. The non-pathogenicity of anti-Dsg3 antibodies in PF can be attributed to the low serum anti-Dsg3 titers, the lack of Dsg3 internalization as detected by IHC, and the absence of PV-associated HLA-DRB1 alleles.

Keywords
Desmoglein 1; Desmoglein 2; Desmoglein 3; Endemic Pemphigus Foliaceus; HLA-DRB1 Chains; Pemphigus

		PF-anti-Dsg3+	PF-anti-Dsg3(-)	PV	p
		n = 16	n = 42	n = 22
Gender	Male	11	26	10	0.296
Gender	Female	5	16	12	0.296
Age (years)	Median (minimum‒maximum)	40.5 (12‒74)^a	32 (12‒71)^b	50 (13‒72)^c	^a·^b0.5361; ^a·^c>0.9999; ^b·^c0.0522
Clinical form	Localized	3	24	NA	0.002
	Generalized	13	11
	U	0	7
Treatment	Treatment-naïve	2	42	22	NA
	No treatment >60 days	1
	Undergoing treatment	12
	U	1
Anti-Dsg1 (UR/L)^#	Median (25%‒75% quartiles)	189.0 (135.1‒254.5)^a	166.9 (121.7‒203.7)^b	40.94 (3.3‒99.4)^c	^a·^b0.4515; ^a·^c<0.0001; ^b·^c<0.0001
Anti-Dsg3 (UR/L)^#	Median (25%‒75% quartiles)	33.1 (23.2‒63.0)^a	0.688 (0.3‒1.6)^b	128.6 (56.6‒168.6)^c	^a·^b0.0002; ^a·^c<0.0001; ^b·^c<0.0001

Patient identification Group PF-anti-Dsg3+	PF-anti-Dsg3+ HLA-DRB1*		PF-anti-Dsg3(-) HLA-DRB1*
2^a	01:02	04:06	01:01	03:02
3	01:01	04:04	01:02	04:11
8	01:01	13:02	01:01	04:08
13^a	01:01	04:01	14:06	16:01
14	01:02	04:05	01:01	04:08
			03:02	15:01
			01:01	04:02
			01:01	04:05
			04:11	15:01
			01:01	07:01
			03:01	08:04
			01:02	11:02
			01:02	15:03
			01:02	04:04
			03:02	16:02
			01:01	15:03

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Factors associated with non-pathogenic antibodies against desmoglein-3 in pemphigus foliaceus☆

Factors associated with non-pathogenic antibodies against desmoglein-3 in pemphigus foliaceus^☆