|
| CASE REPORT |
|
|
|
| Year : 1990 | Volume
: 56
| Issue : 4 | Page : 310-311 |
Epidermolysis bullosa acquisita
V Laxmi Nair, P Ganga
,
Correspondence Address:
V Laxmi Nair
A 26 year old male with epidermolysis bullosa acquisita presenting as severe erosive lesions in the mouth, anterior nares and vesico-bullous lesions over the body arising spontaneously or following trauma is reported. There was no underlying systemic disease and he responded to oral administration of vitamin E.
Keywords: Epidermolysis bullosa acquisita, Mucosal erosions, Vitamin E.
How to cite this article:
Nair V L, Ganga P. Epidermolysis bullosa acquisita. Indian J Dermatol Venereol Leprol 1990;56:310-1 |
A non-hereditary form of epidermolysis bullosa was first described by Colcott in 1897 and Kablitz (1904) was the first to attach the name "acquisita" to it.[1] It is a part of the spectrum of subepidermal blistering diseases. There have been several reports since then. It is clinically characterised by blistering at sites of trauma resulting in scarring and milia. Mucous membrane involvement is variable. Nieboer reported four patients without any mucous membrane involvement.[2] Extensive mucous membrane involvement including oesophageal mucosa has been reported.[3] To the best of our knowledge involvement of nasal mucosa has not been reported earlier. A patient with severe oro-nasal involvement is reported.
| Case Report | |  |
A 26 year old Christian, came for complaints'of severe erosive lesions in the mouth, anterior nares and asymptomatic vesico bullous lesions over the body since 8 months. The lesions started insidiously as intense painful oral erosions. A month later he developed vesico-bullous lesions over the body, arising spontaneously for following trauma.
There were discrete and grouped lesions, some hemorrhagic, distributed asymmetrically over the body, with a predilection for the upper trunk and extremities, particularly the elbows, extensor forearms, hands, feet, palms and soles. The scrotal skin was also affected. Some had a streaky irregular outline. Nikolsky's sign and bulla spreading sign were positive. Minimal atrophy and pigmentary disturbances were evident on healing. Milia were absent.
Though there were episodes of fever and secondary infection, he had no other systemic diseases. However, he had been suffering from chronic urticaria since three years. There was no history of drug intake prior to the onset nor a family history of blistering disease. Haemogram, peripheral smear, urinalysis, blood urea, blood sugar and x-ray chest were normal. Biopsy from an early vesicle showed a subepidermal bulla.
Initially he was managed symptomatically with partial response. He did not respond to dapsone and responded partially to high doses of steroids. Administration of vitamin E, 600 mg. resulted in complete clearance of the lesions.
| Comments | | |
The initial partial response to symptomatic measures may have been due to avoidance of trauma following admission. The presence of irregular streaky blister outline suggested an extrinsic cause like friction. In patients with epidermolysis bullosa acquisita the trauma of rubbing or a slight bump produces blistering.
The development of a blister following self induced trauma, the positive Nikolsky's sign, bulla spreading sign and a subepidermal bulla were in favour of a diagnosis of epidermolysis bullosa acquisita. Cleavage below the PAS positive basement membrane and splitting of the basement membrane is frequently quoted to be seen in EBA, but the basement membrane zone often appears hazy and is of little help in diagnosis[4]. In the absence of immunoflourescence which is not entirely characteristic, a positive Nikolsky's sign and bulla spreading sign helps to differentiate epidermolysis bullosa acquisita from bullous pemphigoid[5]. Trauma of scratching has been reported to produce new lesions in patients with bullous pemphigoid and hence the view that EBA is a variant of bullous pemphigoid in which trauma induced lesions predominate[6]. Others consider it to be separate disorder.[7] Immunoelectronmicroscopy has finally shown it to be a separate disorder distinct from pemphigoid and it is now evident that it is an autoimmune disease.[8]
There is no satisfactory treatment. Systemic steroids control blistering in some. Dapsone and sulphonamides have been reported to be effective. Vitamin E orally was not effective in 4 patients[2] but in our patient the response to vitamin E was dramatic. It appears that vitamin E plays an important role in the stability and integrity of cellular membranes; thus far in man, the only such demonstrated effect is on the RBC.[9]
| References | | |
| 1. | Baxter DL : Epidermolysis bullosa in : Clinical Dermatology, 12th revision, Editor, Demis DJ : Harper and Row publishers, Philadelphia 1985; Vol 2, Unit 6-6, 1-13.  |
| 2. | Nieboer C. Boorsma DM, Woerdeman MJ et al Epidermolysis bullosa acquisita Immunofluorescence, electronmicroscopic and immunoelectronmicroscopic studies in four patients; Brit J Dermatol, 1980; 102 : 383-392. |
| 3. | Yaoita H, Briggaman RA, Lawley TJ et al Epidermolysis bullosa acquisita - Ultrastructural and immunological studies, J Invest Derm, 1981; 76 288-292. |
| 4. | Lever WF and Lever GS : Noninfectious vesicular and bullous diseases, In Histopathology of the skin, 6th ed, JB Lippincott company, Philadelphia, 1983; 92-129. |
| 5. | Srinivas CR, Shenoi SD and Balachandran C : Bulla spreading sign for differentiating epidermolysis bullosa acquisita from bullous pemphigoid, Ind J Dermatol Venereol Leprol, 1989; 55 : 66. |
| 6. | Dahl MGC and Cook LJ : Lesions induced by trauma in pemphigoid, Brit J Dermatol, 1979; 101 : 469-473. |
| 7. | Woodley DJ, Briggaman RA, O'Keefe EJ et al Identification of the skin basement membrane autoantigen in epidermolysis bullosa acquisita, N Eng J Med, 1984; 310 : 1007-1013. |
| 8. | Kero M and Niemi KM. Epidermolysis bulfosa, Int J Dermatol, 1986; 25 : 75-82. |
| 9. | Vanderveen E and Vanderveen JE : Vitamins and nutrients, In : Remington's pharmaceutical sciences, 17th ed, Editor, Gennaro AR : Mack Publishing Company, Easton Pennsylvania, 1985; pp 1008. |
|
|
|
|
|
|
|
|
