title: Aplasia cutis congenita: report of 22 cases.
abstract: BACKGROUND
  Aplasia cutis congenita (ACC) is a rare malformation characterized by absent or scarred areas of skin at birth. Although most commonly found on the scalp, ACC can also involve other locations. Its etiology and pathogenesis remain unclear.
  
  
  OBJECTIVE
  To describe the epidemiologic, clinical, therapeutic, and evolutionary aspects of ACC through a hospital series.
  
  
  METHODS
  We conducted a retrospective study from 1995 to 2012 and reported all cases of ACC.
  
  
  RESULTS
  We enrolled 22 cases (14 girls and eight boys) of ACC during 18 years. The mean age at diagnosis was 5.7 years. Sixteen ACC involved the scalp, five the trunk, and one the left buttock. ACC was oval-shaped in 20 cases, triangular in one case, and linear in one case. The mean size was 4 cm. ACC was associated with bone defects in two cases, various malformations in eight (37.1%), and with syndromic malformation in three (Adams-Olivier syndrome: two cases; Goltz syndrome: one case). Conservative treatment consisting of wound dressing with vaseline was indicated in six cases. Bone reconstruction was performed in two cases. Regular follow-up and no treatment was recommended in 14 cases.
  
  
  CONCLUSIONS
  Our study emphasizes the frequent association of ACC with malformations (37.1%) and bone defects (9%).
authors.affiliation: Department of Dermatology, Hedi Chaker Hospital, Sfax, Tunisia.
authors.forename: Hela
authors.initials: H
authors.lastname: Mesrati
authors.affiliation: Department of Dermatology, Hedi Chaker Hospital, Sfax, Tunisia.
authors.forename: Meriem
authors.initials: M
authors.lastname: Amouri
authors.affiliation: Department of Dermatology, Hedi Chaker Hospital, Sfax, Tunisia.
authors.forename: Hend
authors.initials: H
authors.lastname: Chaaben
authors.affiliation: Department of Dermatology, Hedi Chaker Hospital, Sfax, Tunisia.
authors.forename: Abdelrahmen
authors.initials: A
authors.lastname: Masmoudi
authors.affiliation: Department of Dermatology, Hedi Chaker Hospital, Sfax, Tunisia.
authors.forename: Sonia
authors.initials: S
authors.lastname: Boudaya
authors.affiliation: Department of Dermatology, Hedi Chaker Hospital, Sfax, Tunisia.
authors.forename: Hamida
authors.initials: H
authors.lastname: Turki
mesh_terms: D000293:Adolescent; D000328:Adult; D001842:Bone and Bones / Q000002:abnormalities* / Q000601:surgery; D002081:Buttocks; D002648:Child; D002675:Child, Preschool; D004476:Ectodermal Dysplasia / Q000150:complications* / Q000473:pathology* / Q000628:therapy; D005260:Female; D005489:Focal Dermal Hypoplasia / Q000150:complications; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017880:Limb Deformities, Congenital / Q000150:complications; D008297:Male; D012189:Retrospective Studies; D012536:Scalp Dermatoses / Q000150:complications / Q000151:congenital / Q000473:pathology / Q000628:therapy; D060726:Torso; D055815:Young Adult