Case Number : Case 1804 - 27 April - Dr Arti Bakshi Posted By: Guest

Apologies for late posting today, we had a few technical problems with the site earlier. To make up for it here is a great case from Dr Arti Bakshi.

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http://us13.campaign-archive2.com/?u=ad4b959aee887bce8b61dcb23&id=c71f0aaa69&e=[UNIQID

Geoff Cross - DermpathPRO Projects

Bearing in mind the last 2 fig, I could try the spot of Bullous pemphigoid. The intraepidermal appearance of the blister could be justified with the regenerative epidermal changes...

the distribution is particular could this be related to levamisole? How are the ANCAs? I dont see a clear vasculitis on the pictures but the endothelial cells look quite reactive.

I understood the cocaine abuse was a past history, but maybe am wrong. 

Thought also of BP. 

I first considered this subepidermal blister with eosinophils as bullous pemphigoid, but patient's age and facial lesions militate against that hypothesis. Cocaine snorting can be associated with blistering erythema multiforme features, and the presence of satellite cell necrosis of keratinocytes and the large amount of body surface area involved may point to Stevens-Johnson syndrome / toxic epidermal necrolysis.

I'm agree with Robledo's hypothesis. At first glance without clinic information I was thinking in BP even bites, but with the clinic  I would rather TEN.

Pemphigus Vegetans

12 hours ago, Robledo F. Rocha said:

I first considered this subepidermal blister with eosinophils as bullous pemphigoid, but patient's age and facial lesions militate against that hypothesis. Cocaine snorting can be associated with blistering erythema multiforme features, and the presence of satellite cell necrosis of keratinocytes and the large amount of body surface area involved may point to Stevens-Johnson syndrome / toxic epidermal necrolysis.

Agree

This is a difficult case on H&E alone, so well done all for great d/d.

My thoughts on the H&E section were also along similar lines as the discussion above. There is necrosis of epidermis as well as subepidermal blistering and it is difficult to be sure which is the primary process. However, the adjacent preserved epidermis does not show established interface change, which made me favour a primary subepidermal blister with secondary necrosis. The IMF showed linear pattern of IgA at the dermo-epidermal junction with some C3. IgG was negative.  So,  we went for a Linear IgA disease, which fit clinically as well. Although Linear IgA disease usually shows a neutrophil rich subepidermal blister, rarely eosinophils can predominate. This is particularly true of drug induced cases. Interestingly, a repeat bx done a few days later showed a more typical neutrophil rich subepidermal blister (will ask Geoff to upload pics next week).   Remember, Linear IgA disease can show prominent mucosal involvement and comes in the d/d with mucous membrane pemphigoid with generalised blisters, particularly as the latter can also show IgA deposition (but with IgG).

The cocaine history is probably unrelated but one of the lines of discussion at our CPC meeting was if the patient may be taking other recreational drugs, which may have triggered the disease. (did not come across any reference of this ...as yet!)