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Pregnancy and Delivery with Kindler Syndrome

Background: Kindler syndrome is a rare, autosomal-recessive skin disease characterized by bullous poikiloderma. Mucosal manifestations are common that involve the oral cavity, esophagus, urethra and genital organs. Case Report: A 37-year-old woman with Kindler syndrome received prenatal care at our hospital. Her skin disease did not change during pregnancy and puerperium. Her pregnancy course was uneventful, but an elective cesarean section was performed at 38 weeks of gestation due to vaginal stenosis. Surgical wound healing was uncomplicated. Conclusion: Pregnancy did not exacerbate the cutaneous symptoms of Kindler syndrome in this case. Cesarean delivery may be necessary in cases with severe genital lesions besides obstetrical indications. Careful perioperative management is needed to protect vulnerable skin and mucosa.