PREGNANCY AND DELIVERY IN A PATIENT WITH MUTILATING DYSTROPHIC EPIDERMOLYSIS-BULLOSA (HALLOPEAU-SIEMENS TYPE)

Background: Epidermolysis bullosa dystrophica of the mutilating Hallop eau-Siemens type is a rare inherited skin disease. Those afflicted hav e blisters and pronounced scarring of skin and mucous membranes after minor trauma. Pregnancies are very rare in affected women. Case: A 24- year-old woman, gravida 1, with a severe form of the Hallopeau-Siemens type was monitored closely during pregnancy. The patient spontaneousl y delivered a healthy female neonate at term. Episiotomy wound healing was uncomplicated. Seven months later, she returned in her second pre gnancy, which was complicated by mild anemia and polyhydramnios from p ossible gestational-onset diabetes mellitus. Again, vaginal delivery o f a healthy neonate was performed at term. We did not observe pregnanc y-induced exacerbations of the skin disease. Conclusion: Women with ep idermolysis bullosa dystrophica of the Hallopeau-Siemens type may deci de to have children after careful evaluation of the degree of impairme nt and a thorough explanation of the risks associated with pregnancy a nd delivery. Close monitoring of the pregnant patient is important. Va ginal delivery should be the first choice. Breast-feeding is difficult , but not contraindicated. (C) 1997 by The American College of Obstetr icians and Gynecologists.