Scalp Defect

Aplasia cutis congenita. It is a congenital defect of the scalp characterized by localized loss of the epidermis, dermis, and sometimes, subcutaneous tissue. The defect is present since birth with an incidence of 1 in 10000. Etiology is unknown for most cases` whereas, some are related to genetic syndromes, fetus papyraceous, and teratogens {1}. Most cases are sporadic, some familial cases are reported. The exact mechanism of this disorder is not known. It is hypothesized that aplasia cutis congenital results from incomplete neural tube closure or an embryonic arrest of skin development. Aplasia cutis congenita presents as an asymptomatic ulceration of the scalp at birth that heals with scarring. Lesions from earlier in gestation may present as membrane like scars at birth. Solitary lesion is seen in70 percent of cases, two lesions in 20 percent of cases and three or more lesions in 10 percent of cases, the lesions are non-inflammatory, well demarcated and have variable extent range from 0.5 to 10cm or more {2}. Typically, aplasia cutis congenita is an isolated skin finding. In rare instances, it may be seen with other developmental abnormalities such as skeletal, cardiac, neurologic, or vascular malformations. The diagnosis of aplasia cutis congenita is made by history and physical examination. It should be differentiated from scalp electrode monitors, forceps, or other iatrogenic birthing injuries as well as neonatal herpes simplex virus infection. Skin biopsy reveals an absence of the epidermis and appendageal structures. The prognosis of cutis aplasia congenita as an isolated finding is good. The ulceration typically heals with scarring in a few weeks. The scarred area will persist as an asymptomatic lesion for life {2}.

References:
1. Kane KSM, Lio PA, Stratigos AL. Cutaneous finding in the newborn. In: Kane KSM, Lio PA, Stratigos AL. Color Atlas and Synopsis Of Pediatric Dermatology, Second edition, McGraw Hill Medical, New York. 2009` pp.16-18.
2. Shirvany TE, Zahedpasha Y, Lookzadeh M. Aplasia cutis congenita: a Case Report. Iran J Pediatr. 2009` 19: 185-188
3. Mallory SB, Bree A, Chren P. Aplasia cutis congenita. In: Mallory SB, Bree A, Chren P. Illustrated Manual of Pediatric Dermatology, Taylor and Francis Group London and New York. 2005` pp.17-18

E-published: August 2010 Vol 7 Issue 8 Art # 50