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aplasia_cutis_congenita

Aplasia Cutis Congenita

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by noninflammatory, well-demarcated defects of all skin layers, subcutaneous tissue, with possible co-defects in muscles, periosteum, bone and dura

Although the etiology is still uncertain, a variety of possible causes such as genetic syndromes, teratogens, intrauterine infection with varicella zoster or herpes simplex viruses, fetal exposure to cocaine, heroin, alcohol or antithyroid drugs, infarction of the placenta, and amniotic pathologies are described in the literature.

The incidence is 1 to 3/2000 to 10,000 and 25% of the reported cases are familial with a vast majority (69%) showing an autosomal dominant inheritance or part of a syndrome.

Lesions can be multiple and on different surfaces of the body, but most of the cases have solitary scalp lesions (70%).

It may often occur on the parietal and occipital areas and rarely on extremities.

It can also be associated with other physical anomalies such as defects of eyes, extremities, limbs, gastrointestinal system, genito-urinary system, and central nervous system. 1).

Scalp

Split cord malformation

ACC has seldom been reported to be associated with SCMs 2) 3) 4).

1)
Gazioglu N, Abuzayed B, Aslan H, Saka O, Kafadar AM, Aydin Y. Repair of the venous sinus defect with bovine pericardium dura substitute in a case aplasia cutis congenita. Neurosurg Quart. 2012;22:19–21.
2)
Izci Y, Gönül M, Secer HI, Gönül E. Aplasia cutis congenita: A rare cutaneous sign of split cord malformations. Int J Dermatol. 2007;46:1031–5.
3)
Calikoğlu E, Oztaş P, Yavuzer Anadolu R, Catal F, Görpelioğlu C. Faun tail with aplasia cutis congenita and diastematomyelia. Dermatology. 2004;209:333–4.
4)
Abuzayed B, Erdincler P. Aplasia cutis congenita associated with type I split cord malformation: Unusual case. J Pediatr Neurosci. 2014 Jan;9(1):27-9. doi: 10.4103/1817-1745.131477. PubMed PMID: 24891898; PubMed Central PMCID: PMC4040027.
aplasia_cutis_congenita.txt · Last modified: 2015/08/13 12:40 (external edit)