Treatment of autoimmune blistering diseases during pregnancy

Autoimmune blistering diseases occur when one’s own immune system attacks the skin and mucous membranes (such as your mouth) forming blisters. This group of diseases including pemphigus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, and pemphigoid gestationis can affect females of child-bearing age. Such diseases are especially difficult to treat during pregnancy, since many of the medications normally used can harm the fetus and are contraindicated. Additionally, there is a lack of data and information on medications used during pregnancy, because randomized controlled trials are not typically performed in this setting. However, it is fairly well-established that corticosteroids, which are the mainstay of treatment for these diseases, are considered safe to use during pregnancy.

Topical steroids, such as clobetasol, have not been found to cause any fetal abnormalities and are considered to be a first-line medication. Systemic steroids, while generally safe to use, and usually the medication of choice during pregnancy, can cause increased blood pressure in the mother and increase the risk of gestational diabetes at high doses and with long-term use. Therefore, it is recommended to remain on a dose of 20mg or less during pregnancy, and eventually switch to a steroid-sparing medication, such as azathioprine, once the patient has improved. Topical calcineurin inhibitors, such as topical tacrolimus, are also safe to use, but are likely less efficacious than topical steroids. If a patient is unable to be on systemic steroids, azathioprine is an alternative medication, which has been shown in various studies to be relatively safe to use during pregnancy. Rituximab, which is quickly becoming a front-line treatment for pemphigus, should not be used during pregnancy. However, rituximab is a good option to use months prior to conception, in order to gain control over the disease, as it can allow a patient to go into remission for many months. Cyclosporine has been shown to be efficacious in pemphigoid gestationis and epidermolysis bullosa acquisita, but should only be used if other medications, such as steroids, have failed. Antimalarials, such as hydroxychloroquine and chloroquine, as well as dapsone, are relatively safe during pregnancy, but are rarely used in autoimmune blistering diseases.  Methotrexate, mycophenolate, and cyclophosphamide are absolutely contraindicated during pregnancy and have been well-established as harmful to the fetus.

While pregnant women may be wary of taking medication during pregnancy, more often than not, poor fetal-maternal outcomes occur from poorly controlled disease, rather than from use of a medication. It is important to work with one’s physicians to weigh the risks and benefits of any medication during pregnancy.

Carolyn J. Kushner, Josef Symon S. Concha, Victoria P. Werth
Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

Publication

Treatment of Autoimmune Bullous Disorders in Pregnancy.
Kushner CJ, Concha JSS, Werth VP
Am J Clin Dermatol. 2018 Jun
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