U. Buscher et al., PREGNANCY AND DELIVERY IN A PATIENT WITH MUTILATING DYSTROPHIC EPIDERMOLYSIS-BULLOSA (HALLOPEAU-SIEMENS TYPE), Obstetrics and gynecology, 89(5), 1997, pp. 817-820
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.