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CASE REPORT
Year : 1991  |  Volume : 57  |  Issue : 5  |  Page : 242-243

Congenital absence of skin




Correspondence Address:
C Arun Inamadar


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  Abstract 

A premature baby with extensive and bilaterally symmetrical absence of skin since birth is reported.


Keywords: Aplasia cutis Congenita, Congenital absence of skin.


How to cite this article:
Inamadar C A, Navadgi A, Krishnakumar G, Ya. Congenital absence of skin. Indian J Dermatol Venereol Leprol 1991;57:242-3

How to cite this URL:
Inamadar C A, Navadgi A, Krishnakumar G, Ya. Congenital absence of skin. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2020 Dec 3];57:242-3. Available from: https://www.ijdvl.com/text.asp?1991/57/5/242/3694


Congenital absence of skin otherwise termed as aplasia cutis congenita (ACC) is a circumscribed area in which the skin is absent at birth[1]. The common site of predilection is the midline area of the posterior scalp[2]. We are reporting here a case with more extensive congenital defects of the skin over multiple areas and symmetrical distribution, which is rarely seen. The aetiology, clinical presenta­tion, differential diagnosis and prognosis is discussed. To our knowledge, this is the first report from India of aplasia Cutis congenita affecting multiple areas with symmetrical dis­tribution.


  Case Report Top


A two-day-old premature male baby, born of non-consanguinous marriage was referred to department of Dermatology, by the paediatrician, for opinion regarding baby hav­ing multiple ulcers over the body. On cuta­neous examination, the baby had multiple ar­eas of skin, symmetrically involving temporal area of scalp, trunk, limbs, nose and penis [Figure - 1]. There was complete absence of skin and subcutaneous tissue. The underlying bone, vessels and at places muscle that were visible in these areas were covered with thin glossy appearing tissue. Oral mucous mem­brane was normal. Systemic examination did not reveal any abnormalities. There was no history of development of further lesions af­ter birth till the death on 7th day of life.

Mother's antenatal history was uneventful. There was no family history of similar defect. Hair, mucous membrane and nails of parents did not reveal any obvious abnormalities.


  Comments Top


The diagnosis of congenital absence of skin is primarily clinical. Many theories have been considered for causation of congenital absence of skin, such as, mechanical events, amniotic bands, vascular malformation in utero, failure in the process of embryologic development and cutaneous rupture caused by pressure of cerebral development[3]. It seems reasonable to consider those midline scalp lesion as having an entirely different etiology from those affecting the trunks and limbs symmetrically as with the present case. A familial basis is less common in the latter, and the finding of a monozygotic fetus papyraceus is rather frequent. It has therefore been suggested that this latter type of lesion might be a consequence of embolic derived from blood clots in the dead twin, transmitted via placental anastomoses. In other cases like ours where there is no fetus papyraceus, the emboli might derive from the placenta itself.[1]

Congenital absence of skin may be as­sociated with many other congenital anomolies like-ring constriction of a limb, dis­tal limb anomolies, congenital heart disease, traecheo-esophageal fistula, cleft palate and lip, double cervix and uterus, spastic paraple­gia with mental retardation, cerebral malfor­mation and occult spinal dysraphism[1],[2]

Treatment is rarely necessary as the le­sion almost always heal spontaneously and rapidly, Scalp is the most common site of aplasia cutis congenita, 86 percent of all soli­tary lesions occuring - here[4]. Occasionally, keloidal scarring and joint contracture can be seen. In cases with extensive involvement prevention of secondary infection, further trauma and effective temperature regulation can reduce mortality. Fatality is due to com­plications like haemorrhage, meningitis or in­fection[1]. Mortality in the present case can be explained on the basis of failure of body tem­perature regulation in view of extensive areas of body being devoid of skin in an already deranged thermoregulation due to premature delivery of the baby.

 
  References Top

1.Atherton DJ, Rook A : Naevi and other develop­mental defects, in : Text book of Dermatology, Vol.1, Fourth Ed, Editors, Rook AJ, Wilkinson DS & Ebling FJG: Oxford University Press, Bombay, 1987: P 225-227.  Back to cited text no. 1    
2.Soloman LM, Esterly NB: Neonatal dermatology, Vol IX in the series Major problem in clinical paediatrics, WB Saunders Company Limited, Phila­delphia, 1973; P55  Back to cited text no. 2    
3.Stephan MJ, Smith DW, PonZi JW et al: Origin of Scalp Vertex aplasia cutis, J Paed, 1982; 101:850-853.  Back to cited text no. 3    
4.Frieden IJ: Aplasia cutis congenita: A clinical re­view and proposal for classification, J Amer Acad Dermatol, 1986; 14: 646-660.  Back to cited text no. 4    


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