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Pemphigoid Gestationis

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Autoimmune Bullous Diseases

Abstract

Pemphigoid gestationis (PG) is an autoimmune blistering disease that occurs during pregnancy or soon after delivery. It is possibly caused by cross-reactivity between placental antigens and collagen XVII in the skin. Patients typically present with an intensely pruritic, vesiculobullous rash that starts periumbilically and spreads outwards across the trunk and extremities. Treatment is with immunosuppressive agents; however, given that PG is very rare—estimated to occur in 1 in 50,000 pregnancies—there is a paucity of evidence around specific treatments. Treatment of PG is also complicated by the need to consider the health of the fetus. Often, evidence for the use of immunosuppressant medications in pregnant women for other diseases can be extrapolated to PG. We review the literature and present an algorithm for treatment in the pre- and post-partum periods for women with PG based on the evidence available. We also list areas of focus for the future.

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Abbreviations

ABSIS:

Autoimmune Bullous Skin Disorder Intensity Score

BP:

Bullous pemphigoid

DIF:

Direct immunofluorescence

ELISA:

Enzyme-linked immunosorbent assay

IA:

Immunoadsorption

IIF:

Indirect immunofluorescence

LBW:

Low birth weight

LHRH:

Luteinizing hormone releasing hormone

PDAI:

Pemphigus Disease Area Index

PG:

Pemphigoid gestationis

TSH:

Thyroid stimulating hormone

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Acknowledgements

This material is supported by the Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development.

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Correspondence to Victoria P. Werth MD .

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Moore, E.M., Werth, V.P. (2016). Pemphigoid Gestationis. In: Sami, N. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26728-9_9

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  • DOI: https://doi.org/10.1007/978-3-319-26728-9_9

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