Pregnancy and delivery with Kindler syndrome.

[kindler syndrome]

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 .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 .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 .