Bart’s Syndrome: A Case Report

Bart's syndrome (BS) is a genetic disorder characterized by the absence of localized skin (present at birth), epidermolysis bullosa (EB) and ungueal changes [1] . Clinically Bart's syndrome is associated with


Introduction
Bart syndrome is a rare neonatal pathology combining congenital skin aplasia affecting the extremities and exceptionally described congenital epidermolysis bullosa The purpose of reporting this case is it being rare and how our timely conservative management of skin condition along with nutrition led to the favorable outcome of this non curable congenital disorder, thus avoiding the need for complex surgical management like auto or allografts.

Case Report
Volume 2 -Issue 6

Abstract
Bart's syndrome (BS) is a genetic disorder characterized by the absence of localized skin (present at birth), epidermolysis bullosa (EB) and ungueal changes Feet wounds /ulcers were dressed for twice daily for 5days when they were first washed with normal saline and povidine, followed by application of Fucidin cream which were then covered with chlorhexidine plus paraffin dressing (bactigras) and loose sterile dressings.Rest of the minor skin erosions were cleaned with normal saline followed by Fucidin cream application and were then left uncovered.
Baby was sent home after 5 days with all the necessary home care instructions in detail.Parents were advised to bathe the baby daily with normal saline and skin erosions to be covered with sterile paraffin gauze.Foot dressings were advised to continue daily for 9 days.Follow up was advised at 2 weeks of life.Follow up was done at 2 weeks of life.
Direct immunofluorescence and genetic testing was not done due to unaffordability and unavailability respectively.

Discussion
Bart syndrome is considered exceedingly rare genetic

Conclusion
To achieve the best outcome, care and diagnosis must be accomplished as early as possible [2].Bart's syndrome is an autosomal dominant inheritance caused by mutations in the type VII collagen gene on chromosome 3p3.It was first described in a large family almost half a century ago [3].Bart et al reported the case of 26 members of a family who had congenital absence of skin in the lower limbs, mucous blisters, and absence or deformity of the nails [4].Any part of skin can be involved but disease tends to occur more on extremities and parts exposed to friction and trauma [1].

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disorder.It presents with the classic triad of congenital localized absence of skin over both lower legs, blistering of the skin, and nail dystrophy.The inheritance pattern of Bart syndrome appears to be autosomal dominant but sporadic cases have been reported [2].Congenital localized absence of skin (CLAS) occurs in association with all three major types of inherited epidermal diseases and merits retaining its status as a unique clinical entity [1].The etiopathogenesis of aplasia cutis congenita (ACC) remains unclear.The condition encompasses a heterogeneous group of disorders with or without associated abnormal physical findings, malformation patterns, or genetic diseases [5].Bart's syndrome may also be akin to other anomalies such as pyloric atresia, flattened nose, broad nasal root, and wideset eyes [6,7].Some authors propound it to be caused by intrauterine physical trauma, while others debate that the affected area follows Blaschko's lines [4,8].Cases of aplasia cutis congenita (ACC) secondary to drugs such as methimazole and diclofenac sodium have already been reported.Desloratadine use in pregnancy is associated with hypospadias.In another case described in literature, ACC occurred due to montelukast use [7].The first case of ACC on the leg without association with epidermolysis bullosa was reported in Korea which after conservative treatment with prophylactic topical antibiotics and wet dressing healed with atrophic scar formation and after 2 years there was little scar formation and no functional impairment of the leg [5,9].Journal of Case Reports and Medical History www.acquirepublications.org/JCRMH 4 Histopathology Results

[ 13 ].
Bart syndrome is an odd congenital connection of three distinct skin symptoms-ACC, EBC, and ungeal involvement-whose etiopathogeny is still poorly understood.The prognosis is directly influenced by the management, which is difficult even though the clinical diagnosis is straightforward and aims to treat the skin lesions as effectively as possible while preventing surgery as well as subsequent problems [3].
2 cm.It is wrapped in filter paper and submitted entirely as such in single cassette