Indian Journal of Dermatology
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Year : 2014  |  Volume : 59  |  Issue : 5  |  Page : 527-528
Absence of skin over scalp and short digits in a newborn

University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi, India

Date of Web Publication1-Sep-2014

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DOI: 10.4103/0019-5154.139927

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How to cite this article:
Chhabra N, Kaur R. Absence of skin over scalp and short digits in a newborn. Indian J Dermatol 2014;59:527-8

How to cite this URL:
Chhabra N, Kaur R. Absence of skin over scalp and short digits in a newborn. Indian J Dermatol [serial online] 2014 [cited 2023 Dec 3];59:527-8. Available from:

A 1-day old female child, delivered at thirty six weeks by normal vaginal delivery at home, was admitted in intensive care unit with respiratory insufficiency and crusted lesion over parieto-occipital region of scalp. She was the third child of non-consanguineous parents. On examination, a 4 cm by 3 cm area of scalp defect with overlying scurfing crusts and perilesional atrophic scarring overlying the posterior fontanel was noted [Figure 1]. The underlying skull beneath the scalp defect was normal. Fingers 2-5 on both hands and toes 1-3 of left foot were small with absent nails [Figure 2] and [Figure 3]. Cardiac and neurological examination was normal. There was no family history of scalp or limb defects. The mother denied any history of maternal drug intake, infection or radiation exposure during pregnancy. Hands and feet X-ray of the child revealed hypoplasia of terminal phalanges of fingers and toes. Skull X-ray, chest X-ray, echocardiography, abdominopelvic ultrasonography, brain computed tomography were all normal.
Figure 1: Scalp defect with overlying scurfing crusts and perilesional atrophic scarring overlying the posterior fontanel

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Figure 2: Short fi ngers (2-5) with absent nails involving right hand

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Figure 3: Short toes (1-3) with absent nails involving left foot

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   References Top

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2.McGoey RR, Lacassie Y. Adams-Oliver syndrome in siblings with central nervous system findings, epilepsy, and developmental delay: Refining the features of a severe autosomal recessive variant. Am J Med Genet 2008;146:488-91.  Back to cited text no. 2
3.Koiffmann CP, Wajntal A, Huyke BJ, Castro RM. Congenital scalp skull defects with distal limb anomalies (Adams-Oliver syndrome-Mc Kusick 10030): Further suggestion of autosomal recessive inheritance. Am J Med Genet 1988;29:263-8.  Back to cited text no. 3
4.Jaeggi E, Kind C, Morger R. Congenital scalp and skull defects with terminal transverse limb anomalies (Adams-Oliver syndrome): Report of three additional cases. Eur J Pediatr 1990;149:565-6.  Back to cited text no. 4
5.Borg K, Pfeifle J. Multifocal aplasia cutis congenita, distal limb hemimelia, and cutis marmorata telangiectatica in a patient with Adams-Oliver syndrome. Br J Dermatol 1992;127:160-63.  Back to cited text no. 5
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12.Romaný× J, Puig L, Aznar G, Demestre X, Altirriba O, Alomar A. Adams-Oliver syndrome with unusual central nervous system alterations. Pediatr Dermatol 1998;15:48-50.  Back to cited text no. 12
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  [Figure 1], [Figure 2], [Figure 3]


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