Abstract
The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.
Highlights
Bart syndrome (BS) or aplasia cutis congenita (ACC) type VI, is characterized by congenital localized absence of skin, nail abnormalities and muco-cutaneous blistering lesions[1]
Bruce J Bart in 1966 first described a large kindred with aplasia cutis congenita (ACC) involving lower extremities, blistering lesions of skin and/or oral mucosa, absence or dystrophy of nails and improvement of the blistering process after puberty[1,2,3]
He could not classify the disease due to the unavailability of ultrastructural and immune histochemical studies at that time[4]. Subsequent reports suggested this syndrome to be a subtype of a major form of epidermolysis bullosa[4]
Summary
Bart syndrome (BS) or aplasia cutis congenita (ACC) type VI, is characterized by congenital localized absence of skin, nail abnormalities and muco-cutaneous blistering lesions[1]. Triad of epidermolysis bullosa, congenital absence of skin in lower extremities and absent toe nails. Case report One-day old neonate was transferred from a local hospital to a tertiary care centre for further management of a cutaneous defect involving both lower limbs. He was the first born infant of healthy unrelated parents without similar skin disorders in the family. There were sharply demarcated, glistening red ulcerations involving both lower extremities.
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