Pregnancy and delivery in a patient with mutilating dystrophic epidermolysis bullosa (Hallopeau-Siemens type).

Abstract

Epidermolysis bullosa dystrophica of the mutilating Hallopeau-Siemens type is a rare inherited skin disease. Those afflicted have blisters and pronounced scarring of skin and mucous membranes after minor trauma. Pregnancies are very rare in affected women. A 24-year-old woman, gravida 1, with a severe form of the Hallopeau-Siemens type was monitored closely during pregnancy. The patient spontaneously delivered a healthy female neonate at term. Episiotomy wound healing was uncomplicated. Seven months later, she returned in her second pregnancy, which was complicated by mild anemia and polyhydramnios from possible gestational-onset diabetes mellitus. Again, vaginal delivery of a healthy neonate was performed at term. We did not observe pregnancy-induced exacerbations of the skin disease. Women with epidermolysis bullosa dystrophica of the Hallopeau-Siemens type may decide to have children after careful evaluation of the degree of impairment and a thorough explanation of the risks associated with pregnancy and delivery. Close monitoring of the pregnant patient is important. Vaginal delivery should be the first choice. Breast-feeding is difficult, but not contraindicated.