II. Epidemiology

  1. Incidence: 0.03% of newborns

III. Pathophysiology

  1. Congenital absence of skin
  2. Embryonic defect present in a small subset of patients

IV. Signs

  1. Bulla, ulceration or atrophic lesion

V. Distribution

  1. Most often at the scalp near the vertex

VI. Labs

  1. Biopsy shows absent Dermis or Epidermis

VII. Management

  1. Avoid secondary Bacterial Infection
  2. Surgical excision in some cases

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Ontology: Aplasia Cutis Congenita (C0282160)

Definition (NCI) Localized or widespread congenital absence of skin. The lesions most frequently occur in the scalp, are well demarcated, may be superficial or deep, and are not associated with inflammation.
Concepts Congenital Abnormality (T019)
MSH D004476
ICD10 Q84.8
SnomedCT 35484002, 254237003
Italian Aplasia cutanea congenita
Japanese 先天性皮膚形成不全, センテンセイヒフケイセイフゼン
English CONGENITAL DEFECT OF SKULL AND SCALP, SCALP DEFECT, CONGENITAL, Aplasia Cutis Congenita, ACC, APLASIA CUTIS CONGENITA, NONSYNDROMIC, cutis aplasia, aplasia cutis congenita, aplasia congenita cutis, cuti aplasia, aplasia cutis, congenita aplasia cuti, Aplasia Cutis Congenita, Nonsyndromic, Cutis aplasia of scalp, Aplasia cutis of scalp, Aplasia cutis congenita, Epitheliogenesis imperfecta, Aplasia of skin, Congenital absence of skin, ACC - Aplasia cutis congenita, Cutis aplasia, Aplasia cutis congenita (disorder), Aplasia of skin (disorder), cutis congenita; aplasia, skin; aplasia, aplasia; cutis congenita, aplasia; skin
French Aplasie ectodermique congénitale, Aplasie dermique congénitale, Aplasie cutanée congénitale
German Aplasia cutis congenita
Czech Aplasia cutis congenita, aplasia cutis congenita
Spanish Aplasia Cutánea Congénita, aplasia de cutis congénita (trastorno), aplasia de cutis congénita, aplasia de cutis, aplasia de piel (trastorno), aplasia de piel, epiteliogénesis imperfecta, Aplasia cutánea congénita
Portuguese Aplasia Cutânea Congênita, Aplasia congénita da pele
Hungarian Aplasia cutis congenita
Norwegian Aplasia cutis congenita, Ikke-syndromisk aplasia cutis congenita
Dutch aplasie; cutis congenita, aplasie; huid, cutis congenita; aplasie, huid; aplasie, cutisaplasie, congenitaal, Aplasia cutis congenita