Congenital Absence of Skin (Aplasia Cutis Congenita)

Aplasia cutis congenita (ACC) is a localized congenital absence of skin with a reported incidence of 3 per 10000 live births. Most common location is the scalp. The diagnosis is made clinically. The management of the lesion depends on the size and most of them are managed conservatively but larger lesions need surgical closure. Although aplasia cutis congenita is rare, it is very important for the general pediatrician to recognize this and consider it in the differential diagnosis of skin lesions specially the lesions on the scalp. We present a newborn infant with scalp lesion which was clinically diagnosed as aplasia cutis congenita and was managed conservatively.

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Adams – Oliver Syndrome

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v35i2.12409 ◽

2016 ◽

Vol 35 (2) ◽

pp. 168-171

Author(s):

Avadhesh Joshi ◽

Bhag Singh Karnawat ◽

Manoj Kumar Jangid

Keyword(s):

Congenital Absence ◽

Rare Occurrence ◽

Distal Limb ◽

Aplasia Cutis Congenita ◽

Limb Reduction ◽

Uncommon Disease ◽

Aplasia Cutis

Aplasia cutis congenita is characterized by congenital absence of portion of skin over a localized or widespread area. Adams- Oliver syndrome (subtype-II of ACC) is associated with distal limb reduction anomalies. We describe an infant with this uncommon disease associated with multiple midline lesions, which is a rare occurrence. J Nepal Paediatr Soc 2015;35(2):168-171

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Congenital absence of skin (aplasia cutis congenita)

Plastic & Reconstructive Surgery ◽

10.1097/00006534-197403000-00093 ◽

1974 ◽

Vol 53 (3) ◽

pp. 376

Author(s):

John C. Kelleher

Keyword(s):

Congenital Absence ◽

Aplasia Cutis Congenita ◽

Aplasia Cutis

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NEONATAL APLASIA CUTIS CONGENITA WITH PYOMENINGITIS – A RARE PRESENTATION

10.36106/ijsr/4701852 ◽

2021 ◽

pp. 51-52

Author(s):

Satyendra Satyendra ◽

Jaiprakash Narayan ◽

Jeffy Joy

Keyword(s):

Case Report ◽

Female Child ◽

Congenital Disorder ◽

Congenital Absence ◽

Unknown Etiology ◽

Rare Presentation ◽

Aplasia Cutis Congenita ◽

New Born ◽

Rare Congenital Disorder ◽

Aplasia Cutis

Background - Aplasia cutis congenita is a congenital absence of skin in new born. It can be anywhere over body. Aplasia cutis congenita is a rare congenital disorder of skin. We are presenting a case of aplasia cutis congeni Case report – ta in female child associated with pyomeningitis. Conclusion - Aplasia cutis congenita, its rarity and unknown etiology is the reason of reporting. It is rarely reported a case of Aplasia cutis congenita with association of pyomeningitis.

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Congenital absence of skin (aplasia cutis congenita)

Journal of Pediatric Surgery ◽

10.1016/0022-3468(73)90440-5 ◽

1973 ◽

Vol 8 (5) ◽

pp. 839

Author(s):

Edward J. Berman

Keyword(s):

Congenital Absence ◽

Aplasia Cutis Congenita ◽

Aplasia Cutis

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Aplasia Cutis Congenita of the Scalp with a Familial Pattern

Case Reports in Surgery ◽

10.1155/2016/4264721 ◽

2016 ◽

Vol 2016 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Waleed AlShehri ◽

Sara AlFadil ◽

Alhanouf AlOthri ◽

Abdulaziz O. Alabdulkarim ◽

Shabeer A. Wani ◽

...

Keyword(s):

Congenital Anomalies ◽

Scar Tissue ◽

Congenital Absence ◽

Baby Girl ◽

Aplasia Cutis Congenita ◽

Limb Anomalies ◽

Aplasia Cutis ◽

Familial Pattern

Aplasia Cutis Congenita (ACC) is a condition characterized by congenital absence of skin, usually on the scalp. ACC can occur as an isolated condition or in the presence of other congenital anomalies. Here we describe a case of a 16-day-old baby girl with an isolated ACC of the scalp. Her elder two siblings have been diagnosed with ACC with concomitant cardiac or limb anomalies. The patient was managed conservatively until the defect has formed scar tissue 6 months later.

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