Generalized Epidermolysis bullosa with Congenital Synechiae-Associated Malformations and Unusual Ultrastructure: A New Entity / Reply

V.P. Sybert, Children’s Hospital and Medical Center, 4800 Sand Point Way NE, PO Box C5371, Seattle, WA 98105 (USA) To the Editor I read with interest the case report of Taieb et al. (Dennatologica 1988:176:76-82) on an infant born with eyelid synechiae and areas of erosions on the skin and a cleft palate, which only now came to my attention. I believe this infant fits into the syndrome of AEC (ankyloblepharon filiforme adna-tum, ectodermal dysplasia and cleft lip ± cleft palate). The most characteristic features of this disorder are the adhesions between the eyelids and the clefting defect. In 2 infants with this disorder (also known as Hay-Wells syndrome) we have seen marked erosive changes of the skin which very soon after birth go on to heal. In the second of 2 infants under our care with this condition, a collo-dian membrane was present and peeled at birth leaving areas of erosions. The low-set and unusual ears were also present in both of our patients. The thickening of the stratum corneum is also typical. In summary, I believe that this infant has a recognized autosomal dominant disorder and there is a very low recurrence risk. The cleft palate is part of the disorder and not a fortuitous event.