BACKGROUND: Aplasia cutis congenita (CCA) is a rare developmental anomaly. Its etiology and pathogenesis remain unclear. There are several hypotheses as to the influence of genetic and external factors on the fetus. Terminology of this anomaly is still a subject of discussion. OBJECTIVES: This paper describes 16 patients with CCA of the scalp, ranging in age from 3 to 15 years, observed over the period of 15 years. Among them, girls prevailed 9:7. CCA was more common on the vertex rather then in the temporal region 13:3. In one girl multiple malformations were observed. METHODS: Location, shape, and size of the lesion determined as CCA were analyzed. Detailed history was taken and cytogenetic examination was performed to reveal etiopathogenetic factors. Treatment consisted of excision of the lesion and reconstruction of the scalp with neighboring skin flaps. The excised lesion underwent histologic examination. RESULTS: No etiopathogenetic factors were found. Histopathologic examinations indicate that the observed congenital scalp anomalies resemble a scar. Surgical treatment produced good cosmetic results. 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.
BACKGROUND:Aplasia cutis congenita (CCA) is a rare developmental anomaly. Its etiology and pathogenesis remain unclear. There are several hypotheses as to the influence of genetic and external factors on the fetus. Terminology of this anomaly is still a subject of discussion. OBJECTIVES: This paper describes 16 patients with CCA of the scalp, ranging in age from 3 to 15 years, observed over the period of 15 years. Among them, girls prevailed 9:7. CCA was more common on the vertex rather then in the temporal region 13:3. In one girl multiple malformations were observed. METHODS: Location, shape, and size of the lesion determined as CCA were analyzed. Detailed history was taken and cytogenetic examination was performed to reveal etiopathogenetic factors. Treatment consisted of excision of the lesion and reconstruction of the scalp with neighboring skin flaps. The excised lesion underwent histologic examination. RESULTS: No etiopathogenetic factors were found. Histopathologic examinations indicate that the observed congenital scalp anomalies resemble a scar. Surgical treatment produced good cosmetic results. CONCLUSIONS: Literature on CCA will facilitate physicians to make the right diagnosis. Further accumulation of detailed descriptions on CCApatients may contribute to recognition of etiopathogenesis of this anomaly.