Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach

Fagundes, Patrícia Penha Silveira; Santi, Claudia Giuli; Maruta, Celina Wakisaka; Miyamoto, Denise; Aoki, Valeria

doi:10.1016/j.abd.2020.10.007

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REVIEW • An. Bras. Dermatol. 96 (05) • 2021 • https://doi.org/10.1016/j.abd.2020.10.007 copy

Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach^☆ ☆ How to cite this article: Fagundes PPS, Santi CG, Maruta CW, Miyamoto D, Aoki V. Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach. An Bras Dermatol. 2021;96:581-590. ,^☆☆ ☆☆ Study conducted at the Department of Dermatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

KEYWORDS
Autoimmunity; Epidermolysis bullosa acquisita; Pemphigoid; bullous; Pemphigus; Pregnancy; Skin diseases; vesiculobullous

Disease	Course of the disease during pregnancy	Adverse effects	Neonatal bullous disease
Pemphigus vulgaris	May improve in the third trimester but recur in the postpartum period	Up to 10%, in a series of cases associated with poor disease control. Risk of premature birth in severe illness	Transient and easily managed
Pemphigus foliaceus	Variable course	Minimal	Extremely rare
Bullous pemphigoid	Rarely seen in pregnancy	Not reported	Not reported
Linear IgA Dermatosis	May improve in the second trimester but recur in the postpartum period	Minimal	Not reported
Epidermolysis bullosa acquisita	Extremely rare	A case of exacerbation during pregnancy	One case described
Gestational Pemphigoid	Tends to occur from the second to the third trimester	Increased risk of preterm birth, newborn small for gestational age or underweight	Extremely rare

Authors^a a Bibliographic references are available as supplementary material (Appendix A).	Number of women	Diagnosis before pregnancy	Diagnosis during pregnancy	Disease	Age at onset (years)	Week of gestation at the onset or worsening	Worsening during pregnancy	Improvement during pregnancy	Stable during pregnancy	Worsening in postpartum	Newborn with bullous lesions	Treatment
Lake et al.	1	X		PV	24	NA			X			C/MPM/AZA/IVIG/RTX
	1	X		PV	19	NA			X			C/MPM/IVIG/RTX
Rangel	1		X	PV	24	11	X			X		C/RTX
Elmuradi et al.	1	X		PV	33	24	X					C/MPM/IVIG/RTX
Çayirli et al.	1	X		PV	NA	18	X					C/AZA
Salzberg et al.	1		X	PV	34	6	X					C
Kodagali et al.	1	X		PV	NA	NA					X	C
Solís-Arias et al.	1	X		PV	21	3	X			X		C
Itsukaichi et al.	1	X		PV	34	NA	X			X	X
Ibrahim et al.	1		X	PV	23	22	X				X	C/IVIG
Lorente Lavirgen et al.	1	X		PF	33	24	X				X	C
Drenovska et al.	1		X	PV	27	NA			X	X		C
	1	X		PV	24				X			C
Galarza et al.	1		X	PF	36	17						C
Gushi et al.	1	X		PV	32	NA			X		X	C
Lehman et al.	1	X		PV	32	NA			X
Amer et al.	1	X		PV	NA	NA			X			C
Ugajin et al.	1	X		PV	30	NA	X			X		C
Bonifazi et al.	1		X	PV	NA	NA				PV onset 6 months post-partum	X
Fenniche et al.	1	X		PV	29	12		X		X		C
López-Jornet et al.	1		X	PV	32	20	X					C
Shieh et al.	1		X	PV	29	16	X				X	C
Okubo et al.	1		X	PV	24	18		X		X		C
Hirsch et al.	1	X		PF	23	NA					X
Parlowsky et al.	1	X		PV	NA	NA	X			X
Campo-Voegeli et al.	1		X	PV	35	8	X				X	C
Kalayciyan et al.	1		X	PV	31	32						C/AZA
	1		X	PV	33	8						C
	1		X	PV	32	10						C
	1		X	PV	30	4						C
Muhammad et al.	1		X	PV	28	8	X					C
Masson et al.	1	X		PV	38	12	X					C
Avalos-Díaz et al.	1	X		PF	20	NA	X				X	C
Piontek et al.	1	X		PV	29	12	X					C
Fainaru et al.	1	X		PV	32	13	X					C
Kanwar et al.	1		X	PV	33	24	X				X	C
Hern et al.	1		X	PV	32	14	X					C
	1	X		PV	33	14	X					Symptomatic treatment
Chowdhury et al.	1	X		PV	31	18			X		X	C
Virgili et al.	1		X	PV	27	26	X					C
Tope et al.	1	X		PV	27	NA		X			X	C
Goldberg et al.	1	X		PV	21	28	X					C
Kanwar et al.	1		X	PV	21	6			X			C
Moncada et al.	1	X		PV	28	16			X	X		C
Kaufman et al.	1		X	PF	27	16	X					C
Eyre et al.	1	X		PF	37	NA	X					C
Hup et al.	1	X		PV	20	NA			X
Merlob et al.	1	X		PV	24	NA			X		X	C
Ross et al.	1		X	PV	19	13	X				Fetal death	C/AZA
Wasserstrum et al.	1	X		PV	30	20	X				Fetal death	C/AZA
Green et al.	1	X		PV	36	28	X				Fetal death	C
Moncada et al.	1		X	PV	18	14	X					C
Honeyman et al.	1		X	PV	25	32	X					C
			X	PV	29	NA	NA			X (post-miscarriage)	Miscarriage
			X	PV	33	16	X					C
Terpstra et al.	1		X	PV	23	12	X				Fetal death	C/DAPSONE
Stanoeva et al.	1	X		PV	33	13	X					C
	1		X	PV	28	20			X	X		C
	1		X	PV	29	20			X			C

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[1] ☆
How to cite this article: Fagundes PPS, Santi CG, Maruta CW, Miyamoto D, Aoki V. Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach. An Bras Dermatol. 2021;96:581-590.