Diagnosis: Bullous pemphigoid
Description: Firm blisters
Morphology: Blisters
Site: Abdomen
Sex: F
Age: 77
Type: Clinical
Submitted By: Ian McColl
Differential DiagnosisHistory:
Patient of Dr 77 year old lady admitted to the hospital with blistering skin over a period of a week time. It is mildly itchy. No recent exposure to any triggering factors. She is otherwise well. Not febrile. Past medical history included treated HTN and IHD and her medications include B-blockers, statins, frusemide, Ca supplement. No new medications has been introduced. No recent febrile illness, joint pains, loss of weight or B symptoms. She is otherwise well not febrile and hemodynamically stable. Her routine bloods are all within normal range. Our Geriatrician sent a skin biopsy which was verbally reported to her as likely IgA linear disease or dermatitis herpatiformis. She performed another biopsy from one of the other blisters. She ask me to look at her. The distribution of the blisters is over the abdomen both arms and both legs( mainly extensor aspect). There are scattered erosions as well. Blisters are either clear fluid filled or mildly bloody. The peeled skin of the blisters is very erythmatous. I told our geriatrician that I put my money on bullous Phemphgoid. Clinically it looks typically like it. We sent few bloods and we are waiting for the biopsy result. I suggested high dose steroids and to tapper to lowest possible dose as to maintain good response. She also started her on dapsone. Any suggestions and inputs are highly appreciated. The important thing is the direct immunofluorescence. Was any done? There is a big difference between linear IgA disease and Dermatitis herpetiformis in where the IgA is laid down. (linear in Linear IgA and in the dermal papillae in DH) Bullous pemphigoid would not have IgA deposition. Some of these blisters are tense but others appear to show large erosions. If IgA pemphigus then again the IgA deposition will be between the keratinocytes and be very distinctive. These are not the blisters of DH! DH does not give you erosions like this and the distribution is wrong. Bullous pemphigoid is the commonest blistering disease in the elderly but linear IgA can look similar. You need immunoflorescence here to separate these all out! The oral steroids and Dapsone will cover most possibilities but watch for haemolysis with the Dapsone and check G6DPH levels. (Submitted By: Dr Ian McColl) Thanks very much. I just literally got a call from pathologist while typing this ! Bullous Pemphigoid is confirmed. . Direct Immunoflorescence is positive for IgG . Dapsone has been discontinued.
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