We thank Dr Kanzler for his interesting remarks about our article "Congenital Cutaneous Defects as Complications in Surviving Co-Twins." Despite the lack of electron microscopic and immunomapping studies with our first patient, we think that the correct diagnosis is still ACC type 5, and not Bart syndrome.
First, ACC type 5 is a recognized entity associating multiple, symmetric areas of ACC in a neonate surviving a dead co-twin.1 Also, Bart syndrome is now recognized as a subtype of dominant inherited dystrophic epidermolysis bullosa.2,3 Skin fragility is noticeable even in adulthood. Our patient had no familial history of epidermis bullosa, and she had neither skin fragility nor mucous membrane involvement. At age 7 years, the cutaneous defect caused by neonatal ACC was nearly healed without major scarring or milia (Figure 1). She is now 10 years old and has no sign of epidermis bullosa. Genetic counseling has not been formally requested, but we have indicated that her disorder is sporadic.
Taïeb A, Léauté-Labrèze C. Congenital Cutaneous Defects in Co-Twins—Reply. Arch Dermatol. 1999;135(8):994. doi:10-1001/pubs.Arch Dermatol.-ISSN-0003-987x-135-8-dlt0899
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: