A Case of Aplasia Cutis Congenita, Type VII
2
조회
0
다운로드
2
인용
0
참고문헌
키워드
초록
Aplasia cutis congenita (ACC) is a rare congenital defect in which localized or widespread areas of the skin are absent at birth. In the majority of cases, it is limited to the scalp especially on the vertex although other areas of the body may also be involved. Other congenital malformations can be associated with ACC. We present herein the case of a new born male with unilateral absence of skin on the extensor surface of the right lower leg. There was no associated malformation or skin disease such as blistering or nail abnormailty. According to the classification outlined by Frieden, the condition was diagnosed as type VII aplasia cutis congenita. The treatment of this large ulcer was conservative, wet dressing and prophylactic topical antibiotics. On follow up after 2 years showed that the patient was nearly cured of the ulcer and had only minimal scar formation.
Abstract
Aplasia cutis congenita (ACC) is a rare congenital defect in which localized or widespread areas of the skin are absent at birth. In the majority of cases, it is limited to the scalp especially on the vertex although other areas of the body may also be involved. Other congenital malformations can be associated with ACC. We present herein the case of a new born male with unilateral absence of skin on the extensor surface of the right lower leg. There was no associated malformation or skin disease such as blistering or nail abnormailty. According to the classification outlined by Frieden, the condition was diagnosed as type VII aplasia cutis congenita. The treatment of this large ulcer was conservative, wet dressing and prophylactic topical antibiotics. On follow up after 2 years showed that the patient was nearly cured of the ulcer and had only minimal scar formation.
참고문헌 (19)
- Corden M, "Extrait d'une letter au sujet de trios enfants de la meme nes avee partie des extremities denuee de peau", J Med Chir Pharmacie, 26, pp.556~557
- Frieden IJ, "Aplasia cutis congenital: a clinical review and proposal of classification", J Am Acad Dermatol, 14, pp.646~660 (1986)
- Sybert VP, "Aplasia cutis congenita: a report of 12 new families and review of the literature", Pediatr Dermatol, 3, pp.1~14 (1985)
- Kelly BJ, "Aplasia cutis congenita of the trunk with fetus papyraceus", Pediatr Dermatol, 19, pp.326~329 (2002)
- Mannino FL, "Benirschke: Congenital skin defects in fetus papyraceus", J Pediatr, 4, pp.559~564 (1997)
- Fusi L, "Acute twin-twin transfusion: A possible mechanism for brain-damaged survivors after intrauterine death of a monochronic twin", Obstet Gynecol, 78, pp.517~520 (1991)
- Torpin R, "Amniochronic mesoblastic fibrous strings and amniotic bands", Am J Obstet Gynecol, 91, pp.65~75 (1965)
- Light TR, "congenital constriction band syndrome. Pathophysiology and treatment", Yale J Biol Med, 66, pp.143~155 (1993)
- Weedon D, "Skin pathology" (1997), Churchill-Livingstone
- Bridges AG, "Pretibial dystrophic epidermolysis bullosa", Cutis, 63, pp.329~332 (1999)
- Mempel M, "The wide spectrum of clinical expression in Adams-Oliver syndrome; a report of two cases", Br J Dermatol, 104, pp.1157~1160 (1999)
- Gutzmer R, "Bart syndromeseparate entity or a variant of epidermolysis bullosa", Hautarzt, 48, pp.640~644 (1997)
- Gharpuray MB, "Congenital localized absence of skin associated with blistering of the skin and mucous membrane; Bart's syndrome", Cutis, 44, pp.318~320 (1989)
- Park SH, "A case of aplasia cutis conge", Korean J Dermatol, 22, pp.346~349 (1984)
- Choi HM, "Two cases of aplasia cutis congenita", Korean J Dermatol, 23, pp.83~87 (1985)
- Chung J, "Aplasia cutis congenita", Korean J Dermatol, 32, pp.698~702 (1994)
- Park JM, "A case of aplasia cutis congenita", Korean J Dermatol, 38, pp.295~297 (2000)
- Mo HJ, "A case of aplasia cutis congenita", Korean J Dermatol, 39, pp.612~614 (2001)
- 박 근, "임신 초기에 Methimazole 노출 후 발생한 선천성 피부 형성 부전증 1예", 대한피부과학회지, 44(5), pp.642~644 (2006), 대한피부과학회