BART SYNDROME: CONGENITAL APLASIA CUTIS TYPE VI

Main Article Content

Sobia Rubab
Madiha Naz
Nusrat Hussain Buzdar
Faiza Anam

Abstract

Bart’s syndrome is described as absence of skin in a localized area by birth also called Aplasia cutis congenital type VI. It may be concomitant with any type of EB but the most common cases reported are allied with dystrophic epidermolysis bullosa (DEB dominant). The clinical presentation of this disease is that the areas of the body which are prone to injury area lacking skin e.g. hands and feet. Diagnosis is done by clinical examination and but for proper disease classification microscopic examination is required only symptomatic treatment is done.


Our case is of a neonate who presented with wide-denuded erythematous areas on the neck, upper and lower limbs. Clinical examination helped us to reach the final diagnosis that is Bart’s syndrome.

Article Details

How to Cite
1.
Sobia Rubab, Naz M, Nusrat Hussain Buzdar, Faiza Anam. BART SYNDROME: CONGENITAL APLASIA CUTIS TYPE VI. J Postgrad Med Inst [Internet]. 2023 Mar. 1 [cited 2024 Nov. 18];37(1):76-8. Available from: https://jpmi.org.pk/index.php/jpmi/article/view/3068
Section
Case Report
Author Biography

Faiza Anam, Department of Dermatology, Nishter Medical University, Multan, Punjab- Pakistan.

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References

Bart BJ, Gorlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormali¬ties resembling epidermolysis bullo¬sa. A new syndrome. Arch Dermatol. 1966;93(3):296-304.

Al-Gburi S, Namuq Z. Twin Neo¬nates With Bart's Syndrome. Cureus. 2022;14(1):e21363. DOI:10.7759/cu¬reus.21363.

Sigha OB, Mbono Betoko R, Nkoro GA, Fossi Happi M, Ekoube CE, Kelbaba BB, et al. Bart's syndrome associat¬ed with a disorder of sexual differen¬tiation: An atypical presentation in a Cameroonian newborn. Clin Case Rep. 2022;10(1):e05234. DOI:10.1002/ ccr3.5234.

Alfayez Y, Alsharif S, Santli A. A Case of Aplasia Cutis Congeni¬ta Type VI: Bart Syndrome. Case Rep Dermatol. 2017;9(2):112-18. DOI:10.1159/000478889.

Bajaj DR, Qureshi A. Bart’s syndrome: a case report. J Pak Assoc of Dermatol. 2008;18(2):113–5.

Rajpal A, Mishra R, Hajirnis K, Shah M, Nagpur N. Bart's syndrome. Indi¬an J Dermatol. 2008;53(2):88-90. DOI:10.4103/0019-5154.41655.

Duran McKinster C, Rivera Franco A, Tamayo L, de la Luz Orozco Covarrubias M, Ruiz Maldonado R. Bart syndrome: the congenital localized absence of skin may follow the lines of Blaschko. Report of six cases. Pediatr Dermatol. 2000;17(3):179-82. DOI:10.1046/ j.1525-1470.2000.01747.x.

Sigha OB, Mbono Betoko R, Nkoro GA, Fossi Happi M, Ekoube CE, Kelbaba BB,. Bart's syndrome associated with a disorder of sexual differentiation: An atypical presentation in a Cameroo¬nian newborn. Clin Case Rep. 2022 Jan 7;10(1):e05234. DOI:10.1002/ ccr3.5234.