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Year : 2000  |  Volume : 66  |  Issue : 6  |  Page : 310-311

Generalised Non Lethal Junctional Epidermolysis Bullosa

Correspondence Address:
Vishaal Madan

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Source of Support: None, Conflict of Interest: None

PMID: 20877112

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An 18-year olf boy presented with multiple bullae and atrophic depigmented lesions over the acral region. On examination he also had dystrophic and absent nails, discolored and dystrophic teeth and complaints of gradual decrease in hearing ability and dysphagia. No family history of similar lesions was present.

Keywords: Epidermolysis bullosa non lethal junctional

How to cite this article:
Madan V, Gupta U. Generalised Non Lethal Junctional Epidermolysis Bullosa. Indian J Dermatol Venereol Leprol 2000;66:310-1

How to cite this URL:
Madan V, Gupta U. Generalised Non Lethal Junctional Epidermolysis Bullosa. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2020 Jun 5];66:310-1. Available from: http://www.ijdvl.com/text.asp?2000/66/6/310/4957

  Introduction Top

Epidermolysis bullosa comprises of a group of genetically determined skin fragility disorders characterized by blistering of the skin and mucosa following mild mechanical trauma. Junctional epidermolysis bullosa is a term characterized by blistering wherein the basic pathology is in the lamina lucida.[1],[2] Junctional epidermolysis bullosa is conventionally divided into two main categories, Herlitz lethal) and non- Herlitz (non-lethal). The clinical course may be similar to the Herlitz form of junctional epidermolysis bullosa with generalised skin fragility and blistering but the patient usually survives to adulthood.[3],[5]

  Case Report Top

An 18-year-old boy was referred from the ENT Department where he was being investigated for obstruction in the passage of all kinds of food. He was having bullous eruptions over the acral areas with atrophic depigmented scars.

One month after his birth, the patient had started developing vesicular lesions over the body parts exposed to trauma. These gave way to bullous lesions which finally ruptured leaving depigmented atrophic scars. The teeth were discolored and dystrophic but they were normal till the temporary set of teeth were shed. At the age of 10 the nails were gradually shed and now no nails were present on the fingers or toes. In the past six years his hearing ability had decreased and since two years obstruction to all kinds of food had been a major cause of discomfort to the patient.

Routine investigations were carried out and found to be normal. Oesophagoscopy could not be performed, as the pharyngeal passage was narrow and bled profusely on passage of the oesophagoscope. X- Ray of the chest revealed no abnormality. Audiometry was suggestive of middle ear deafness, and histopathology confirmed the diagnosis of epidermolysis bullosa.

  Discussion Top

Generalized non- lethal junctional epidermolysis bullosa (syn. epidermolysis bullosa atrophicans generalisata mitis, Generalised atrophic benign epidermolysis bullosa, GABEB.) has its course similar to the gravis/Herlitz from of the disease, though survival to adulthood is a major feature. Presence of blisters since infancy and atrophic scarring with dystrophic and absent nails, oesophageal dysphagia, and hypoacousis,[3],[6] suggested the diagnosis. Though alopecia is considered a major sign of this disease, it was absent in this patient.

  References Top

1.Gedde- Dahl.T, Jr Anton- Lain precht I. Epidermolysis bullosa. In Emery AE H, Rimoin DL, eds. Principles and Practice of Medical Genetics. London: Churchill Livingstone, 1983; 672- 687.  Back to cited text no. 1    
2.Eady RAI, McGrath JA, McMillan JR. Ultrastructural clues to genetic disorders of skin: the dermal-epidermal junction. J Invest Dermatol 1994; 103: 13s- 18s.  Back to cited text no. 2    
3.Hintner H, Wolff K. Generalized atrophic benign epidermolysis bullosa. Arch Dermatol 1982; 118: 375- 84.  Back to cited text no. 3    
4.Paller AS, Fine J-D, Kaplan 5, et al. The generalised atrophic benign form of junctional EB: experience with four patients. Arch Dermatol 1986; 122: 704- 710.  Back to cited text no. 4    
5.Foldes. C, Wallach D, Aubiniere E, et al. Generalised atrophic benign form of Junctional EB. Dermatologica 1988; 176: 83- 90.  Back to cited text no. 5    
6.Hashimoto I, Schnyder UW, Anton Lanprecht I. Epidermolysis bullosa hereditaria with junctional blistering in an adult. Dermatologica 1976;152:72-86.  Back to cited text no. 6    


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