APLASIA-CUTIS-CONGENITA OF THE SCALP - REPORT OF 16 CASES

BACKGROUND. Aplasia cutis congenita (CCA) is a rare developmental anom aly. Its etiology and pathogenesis remain unclear. There are several h ypotheses as to the influence of genetic and external factors on the f etus. Terminology of this anomaly is still a subject of discussion. OB JECTIVES. This paper describes 16 patients with CCA of the scalp, rang ing in age from 3 to 15 years, observed over the period of 15 years. A mong them, girls prevailed 9:7. CCA was more common on the vertex rath er then in the temporal region 13:3. In one girl multiple malformation s were observed. METHODS. Location, shape, and size of the lesion dete rmined as CCA were analyzed. Detailed history was taken and cytogeneti c examination was performed to reveal etiopathogenetic factors. Treatm ent consisted of excision of the lesion and reconstruction of the scal p with neighboring skin flaps. The excised lesion underwent histologic examination. RESULTS. NO etiopathogenetic factors were found. Histopa thologic examinations indicate that the observed congenital scalp anom alies resemble a scar. Surgical treatment produced good cosmetic resul ts. CONCLUSIONS. Literature on CCA will facilitate physicians to make the right diagnosis. Further accumulation of detailed descriptions on CCA patients may contribute to recognition of etiopathogenesis of this anomaly. (C) 1998 by the American Society for Dermatologic Surgery, I nc.