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LETTER TO EDITOR
Year : 1992  |  Volume : 58  |  Issue : 2  |  Page : 135-136

Bart's syndrome




Correspondence Address:
M B Gharpuray


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How to cite this article:
Gharpuray M B, Tolat S N. Bart's syndrome. Indian J Dermatol Venereol Leprol 1992;58:135-6

How to cite this URL:
Gharpuray M B, Tolat S N. Bart's syndrome. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 Mar 28];58:135-6. Available from: http://www.ijdvl.com/text.asp?1992/58/2/135/3775


To the Editor,

We write in reference to an article entitled "Congenital absence of skin" by Anil Navadgi et al published in the Ind J Dermatol Venereol Leprol 1991; 57: 242-3.

We would like to share our experience with one such similar case.

A new born infant was brought to us for extensive areas of absent skin, involving both lower extremities [Figure - 1]. We diagnosed the baby as having, "aplasia cutis congenita". Within a month or so, the skin defects healed with thin atrophic scars but to our surprise 2 months later, our patient started developing blisters on her scalp and trunk [Figure - 2]. The oral mucus membranes also developed erosions.

We now revised our diagnosis from aplasia cutis congenita to "Bart's syndrome". Bart's syndrome is one of the varieties of the mechanobullous diseases or epidermolysis bullosa. The syndrome is characterized by localized absence of skin, blistering and nail dystrophy. [1] The cutaneous blisters eventually heal with scarring.

It is unfortunate and confusing that the authors of the present article have used the term "congenital absence of skin" as synonymous with "aplasia cutis congenita". It should be appreciated that congenital absence of skin over extensive areas can be a feature of the Bart's syndrome [1],[2] and the Herlitz's disease; [1] apart form "aplasia cutis congenita". Both the Bart and Herlitz syndromes are varieties of the mechanobullous diseases.

In view of the early fatal outcome of the reported case, which is a feature of Herlitz's disease, we would suggest an alternative diagnosis of Herlitz disease for the reported case.

 
  References Top

1.Bart B J, Gorlin R J, Anderson E V, et al. Congenital absence of skin and associated abnormalities resembling epidermolysis bullosa. Arch Dermatol 1966; 93: 296.  Back to cited text no. 1    
2.Lever W F, Schaumburg- Lever G. Congenital diseases, Aplasia cutis congenita. In: Histopathology of the skin. 7th edn. Philadelphia: J B Lippincott, 1990; 75.  Back to cited text no. 2    


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[Figure - 1], [Figure - 2]



 

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Online since 15th March '04
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