|Year : 2001 | Volume
| Issue : 6 | Page : 329
Aplasia cubs congenmta associated with meningomyelocoel
Sunil K Gupta , Devinder K Jasuja , Satish K Pandit
Department Skin and VP, Pt. B.D.S. PGIMS, Rohtak, India
33-L, Model Town, Rohtak, India
Aplasia cutis congenita is a rare congenital anomaly where skin is missing over part of the body. Most of the cases are present on the scalp. We present a case of ACC where skin was absent over the lumbosacral area. It was associated with meningomyelocoel.
|How to cite this article:|
Gupta SK, Jasuja DK, Pandit SK. Aplasia cubs congenmta associated with meningomyelocoel. Indian J Dermatol Venereol Leprol 2001;67:329
|How to cite this URL:|
Gupta SK, Jasuja DK, Pandit SK. Aplasia cubs congenmta associated with meningomyelocoel. Indian J Dermatol Venereol Leprol [serial online] 2001 [cited 2019 Dec 12];67:329. Available from: http://www.ijdvl.com/text.asp?2001/67/6/329/11248
| Introduction|| |
Aplasia cutis congenita is a descriptive term used to designate a localised area of congenital absence of skin. As an isolated defect, aplasia cutis congenita (ACC) is most commonly represented by a small, circular, midline lesion near the vertex of the scalp. The present case is reported because of the rarity of the condition and its association with meningomyelocoel.
| Case Report|| |
A 7-day- old, full term, normally delivered, male infant, weighing 2.8 Kgs was presented to the Skin Out Patient Department with complaint of abnormal skin over the back. The other sibling was normal and there was no family history of skin defects, no history of febrile illness, drug ingestion or exposure to X-rays during pregnancy. Examination of baby revealed 6"X3" cutaneous defect at the lumbosacral region. A lump was present which was found to be meningomyelocoel. There was sensorineural deficit in the lower limbs. Faecal incontinence was present. Presence of supernumerary digit on the left hand was a chance finding. Ultrasonography of abdomen and skull for ventricles and echocardiography were within normal limits. There was no other apparent congenital anomaly.
| Discussion|| |
Most of the cases of the congenital skin defects are found on the scalp. Less commonly, ACC presents in other body areas, in which case the defects are usually symmetrically distributed over the extensor surfaces of the individual. The first case of ACC was reported by Cordon in 1767 in sisters, where skin involvement was limited to the lower extremities. [Figure - 1] Five cases of ACC reported by Higginbottom et al, had congenital skin defects in the lumbosacral region and were associated with occult spinal dysraphism, whereas one case was associated with meningomyelocoel and sensorineural deficit in the lower limbs. This case probably represents type-4 of ACC where there is congenital absence of skin overlying developmental malformations.
| References|| |
|1.||Mannino FL, Jones KL, Benirschke K. Congenital skin defects and fetus papyraceus. J Pediatr 1977;91:559. [PUBMED] |
|2.||Cordon M. Au fujet de trois enfans de la meme mere,nes avec une partie des extremities aunuee de peau. J de Med de Chir Parm ect. 1767; 3 :2183. |
|3.||Higginbottom MC, Jones KL, James HE, et al. Aplasia cuits congenita :A cutaneous marker of occult spinal dysraphism. J Pediatr 1980;96:627-629. |
|4.||Atherton DJ. Naevi and other developmental defects. In : Textbook of Dermatology. Edited by Rook A, Wilkinson DS, Ebiling FJG. 6th edn, Oxford university Press 1998; 519-616. |