Scalp erosion in ankyloblepharon-ectodermal defect-cleft lip and/or palate (AEC syndrome): treatment with acellular dermal matrix.
Ankyloblepharon-ectodermal defect-cleft lip and/or palate (AEC syndrome, also known as Hay-Wells syndrome) is an autosomal dominant disease caused by mutation in the p63 gene that is primarily characterized by facial clefting, presence of ankyloblepharon, ectodermal dysplasia, and scalp erosion. Scalp erosion is perhaps the most debilitating manifestation of AEC due to its problematic treatment that is fraught with failure given the underlying pathology of the p63 mutation causing dysfunctional wound healing. Management is often targeted in a stepwise fashion, beginning with daily baths, light debridement, and emollients and progressing to extensive skin excision. Skin grafting has limited success and, inevitably, infections requiring aggressive debridement and antibiotic therapy result from dysfunctional healing. The use of acellular dermal matrix for treatment of scalp erosion is a novel approach attempted in a patient with severe scalp disease. Here we report her case and the failure of treatment, along with possible explanations and suggestions for future therapy.
Department of Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA 90027, USA.
Clifford
C
Sheckter
Elizabeth
E
Rommer
Cameron
C
Francis
Vanessa
V
Block
Joyce
J
Chen
Murtaza
M
Rizvi
Mark M
MM
Urata
Jeffrey
J
Hammoudeh
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001458:Bandages; D002809:Chondroitin Sulfates / Q000627:therapeutic use*; D002971:Cleft Lip / Q000473:pathology* / Q000628:therapy*; D002972:Cleft Palate / Q000473:pathology* / Q000628:therapy*; D003094:Collagen / Q000627:therapeutic use*; D003646:Debridement; D004476:Ectodermal Dysplasia / Q000473:pathology / Q000628:therapy*; D004643:Emollients / Q000627:therapeutic use; D005124:Eye Abnormalities / Q000473:pathology* / Q000628:therapy*; D005143:Eyelids / Q000002:abnormalities* / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D012535:Scalp / Q000473:pathology*; D007507:Therapeutic Irrigation
D000900:Anti-Bacterial Agents; D004643:Emollients; D005938:Glucocorticoids; C114835:integra artificial skin; D002809:Chondroitin Sulfates; D003094:Collagen