A systematic review of 111 pemphigus vulgaris patients during pregnancy found that disease control before and during pregnancy is the most important factor predicting maternal health, gestational complications, and neonatal outcomes.
- Pemphigus vulgaris during pregnancy should be considered high-risk, requiring close monitoring
- Disease control and remission before pregnancy significantly improves outcomes for mother and baby
- Neonatal pemphigus occurred in 38% of cases, fetal mortality was 9.8%, and post-partum flares in 37%
How this compares to prior research
Pemphigus vulgaris is a rare autoimmune blistering disease that creates unique challenges during pregnancy due to the complex interplay between hormonal and immune systems. Previous understanding recognized pregnancy as a high-risk situation for women with autoimmune diseases, but comprehensive data on pemphigus vulgaris specifically during pregnancy has been limited. This systematic review represents the first comprehensive analysis of outcomes across 90 studies, providing clearer guidance on disease management and expected outcomes.
Pemphigus vulgaris prevalence trends, global data, 1980–1989
Key findings
- Analysis of 90 studies involving 111 pregnant pemphigus vulgaris patients showed that clinical and serological disease control is the most important factor influencing maternal health and fetal outcomes
- Neonatal pemphigus occurred in 38% of cases, fetal mortality was 9.8%, and post-partum disease flares occurred in 37% of patients, all correlating with lack of maternal disease control during pregnancy
- Systemic corticosteroids remain the primary treatment, with azathioprine appearing to be the safest immunosuppressive agent and intravenous immunoglobulin providing significant benefits when available
What this means in practice
- Plan pregnancy when pemphigus vulgaris is in remission to reduce risks to mother and baby
- Expect frequent monitoring throughout pregnancy and post-partum if you have pemphigus vulgaris
- Discuss topical therapy options with your doctor to potentially reduce need for higher corticosteroid doses
Frequently asked questions
Can women with pemphigus vulgaris have a successful pregnancy?
Yes, pemphigus vulgaris does not preclude successful pregnancy. The best outcomes occur when the disease is in remission before conception and well-controlled throughout pregnancy.
What are the risks to the baby if the mother has pemphigus vulgaris?
Neonatal pemphigus occurs in 38% of cases and fetal mortality is 9.8%. These risks are significantly higher when maternal disease is not well-controlled during pregnancy.
What medications are safe for treating pemphigus vulgaris during pregnancy?
Systemic corticosteroids are the mainstay treatment. Azathioprine appears to be the safest immunosuppressive agent, and intravenous immunoglobulin can provide benefits when available and affordable.
Key terms explained
Pemphigus vulgaris
A rare autoimmune disease causing painful blisters on skin and mucous membranes, including the mouth
Neonatal pemphigus
Temporary blistering condition in newborns caused by maternal antibodies crossing the placenta during pregnancy
Immunosuppressive agent
Medication that reduces immune system activity to prevent it from attacking the body\’s own tissues
Source: Critical analysis of pemphigus vulgaris in pregnancy. · DOI: doi: 10.3389/fimmu.2026.1788979