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   Introduction
   Case Report
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CASE REPORTS
Year : 1999  |  Volume : 65  |  Issue : 6  |  Page : 296-297

Epidermolysis bullosa acquisita in a young female




Correspondence Address:
Reena Rai


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Source of Support: None, Conflict of Interest: None


PMID: 20921694

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How to cite this article:
Rai R, Srinivas C R, Mustafa M, Surendra. Epidermolysis bullosa acquisita in a young female. Indian J Dermatol Venereol Leprol 1999;65:296-7

How to cite this URL:
Rai R, Srinivas C R, Mustafa M, Surendra. Epidermolysis bullosa acquisita in a young female. Indian J Dermatol Venereol Leprol [serial online] 1999 [cited 2019 Jun 20];65:296-7. Available from: http://www.ijdvl.com/text.asp?1999/65/6/296/4847





  Introduction Top


Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal blistering disease in which antibodies to type VII collagen are found. Flaccid, serous or haemorrhagic bulla develops at the sites of trauma and heal with scarring, milia and hyperpigmentation. Most patients are over the age of fifty, although EBA has beeen reported in children.[1]


  Case Report Top


A 20-year-old female developed continuous cough with expectoration, loss of appetite and weight. She was diagnosed as having pulmonary tuberculosis and referred for bullous lesions since 5 years.

The bulla was tense and both Nikolsky's sign and 'bulla spread' sign were positive. In addition areas of scarring, milia and post inflammatory hyperpigmentation were seen [Figure - 1]

The patient developed haematemesis, and endoscopy revealed a longitudinal ulcer in the oesophagus. Biopsy of an early bulla showed a sub-epidermal bulla, filled with RBCs and transudate. Direct Limmunofluorescence showed a band of IgM, IgA, C3 and fibrin at the basement membrance zone. Indirect immunofluorescene (IIF) showed fluorescence at the floor of the split skin.


  Discussion Top


The diagnosis of EBA was made by the history of repeated episodes of bullous lesions which healed with scarring and milia. The presence of 'bulla spread' sign as observed in our patient has already been reported.[2] IIF on saline split skin helped to confirm the diagnosis by showing immunofluorescence along the floor of the split skin,[3] and thus ruled out bullous pemphigoid where the band is seen along the roof of the split skin. We report this case as this is the first confirmed case of EBA in India in the younger age group.



 
  References Top

1.Rubenstein R, Esterly NB, Fine JD. Childhood epidermolysis bullosa acquisita. Detection in a 5-year-old girl. Arch Dermatol 1987;772-776.  Back to cited text no. 1    
2.Srinivas CR, Balachandran C, Wojnarowska F, et al. Epidermolysis bullosa aquisita with positive immunoblotting on split skin and bulla spreading sign. Indian J Dermatol Venereol Leprol 1991;57:287-288.  Back to cited text no. 2    
3.Gammon WR, Briggaman RA, Inman AO, et al. Differentiating anti-lamina lucida and anti sublamina densa anti BMZ antibodies by indirect immunofluorescence on 1.0M sodium chloride separated skin. J Invest Dermatol 1984;82:139-144.  Back to cited text no. 3    


    Figures

[Figure - 1]



 

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