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June 1993, Vol 147, No. 6 >
Article | June 1993

Picture of the Month FREE

Alexis J. Carras; Bernard A. Cohen, MD; Walter W. Tunnessen, MD
[+] Author Affiliations

Accepted for publication September 15, 1992.

Reprint requests to The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104 (Dr Tunnessen).


Am J Dis Child. 1993;147(6):689-690. doi:10.1001/archpedi.1993.02160300095033.
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Denouement and Discussion 

Aplasia Cutis Congenita 

Definition  Aplasia cutis congenita is a heterogeneous group of disorders characterized by localized or widespread areas of absent skin at birth. More than 500 cases of this disorder have been reported since the first description in 1767.1

Manifestations  The lesions of aplasia cutis congenita vary in appearance depending on when they occur during intrauterine development, the depth of involvement, and the size and location of the lesions. Those occurring early in gestation may heal before delivery and appear as areas of atrophy, fibrosis, and alopecia. Defects that develop close to delivery usually appear ulcerated or covered by a thin, glistening membrane at birth. Superficial lesions are associated with minimal scarring and hair loss.The size of the lesions can vary considerably from 0.5 cm2 to 100 cm2 2,3 The lesions may occur on any body surface, although most defects appear on the head and 70% to 75% occur as a solitary lesion.4

REFERENCES

1 +
Frieden IJ.  Aplasia cutis congenita: a clinical review and proposal for classification . J Am Acad Dermatol. 1986;;14:646-660.
2 +
Scribanu N, Temtamy SA.  The syndrome of aplasia cutis congenita with terminal transverse defects of limbs . J Pediatr. 1975;;87:79-82.
3 +
Farmer AW, Maxmen MD.  Congenital absence of skin . Plast Reconstr Surg Transplant Bull. 1960;;25:291-297.
4 +
Ingalls NW.  Congenital defects of the scalp, III: studies in the pathology of development . Am J Obstet Gynecol. 1933;;25:861-873.
5 +
Demmel U.  Clinical aspects of congenital skin defects . Eur J Pediatr. 1975;;121:21-50.

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References

1 +
Frieden IJ.  Aplasia cutis congenita: a clinical review and proposal for classification . J Am Acad Dermatol. 1986;;14:646-660.
2 +
Scribanu N, Temtamy SA.  The syndrome of aplasia cutis congenita with terminal transverse defects of limbs . J Pediatr. 1975;;87:79-82.
3 +
Farmer AW, Maxmen MD.  Congenital absence of skin . Plast Reconstr Surg Transplant Bull. 1960;;25:291-297.
4 +
Ingalls NW.  Congenital defects of the scalp, III: studies in the pathology of development . Am J Obstet Gynecol. 1933;;25:861-873.
5 +
Demmel U.  Clinical aspects of congenital skin defects . Eur J Pediatr. 1975;;121:21-50.

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