Location
Philadelphia, PA
Start Date
3-5-2023 1:00 PM
End Date
3-5-2023 4:00 PM
Description
Introduction: Bullous Pemphigoid (BP) is an autoimmune blistering condition that results from formation of autoantibodies against basement membrane hemidesmosome proteins, specifically BP180 and BP230. Classic BP presents as pruritic, tense blisters on the trunk and extremities, most commonly in the elderly. BP’s pathophysiology involves IL4/IL5/IL13 and eosinophils. Dupilumab is an IgG monoclonal antibody targeted at the IL4-α receptor that works to attenuate the IL4/IL13 pathway involved in T2 differentiation. Dupilumab has clinical applications for T2 mediated disease and shows promise in the therapy of BP.
Case Description: We report a case of an 83-year-old Asian male who presented in the spring of 2019 with a chief complaint of blistering lesions accompanied by significant pruritus. Medical history was positive for hypertension controlled on amlodipine and losartan and hypercholesterolemia not on any medical therapy. Past medical history and family history were negative for any autoimmune or skin disorders. Tense bullae were noted in the arm, hands, thighs, feet, groin and the head. The blistering rash appeared to be urticarial lesions which were unresponsive to prednisone therapy. Hypersensitivity reaction and drug eruption were suspected.
Initial biopsy revealed spongiotic dermatitis with eosinophilic spongiosis and there was suggestion of BP. A diagnosis of BP was later confirmed on a second biopsy via direct immunofluorescence.
Initial treatment with oral prednisone and doxycycline 100mg bid, topical 0.1% triamcinolone bid and topical 1% silver sulfadiazine was unsuccessful. Every time prednisone was tapered the patient would have flares of new blisters. Sarna was used for managing pruritus. Mycophenolate mofetil (1g BID) and dapsone (25 mg BID and eventually 2x25mg tablets, BID) were attempted but gradually discontinued because of significant neuropathy and paresthesias. 5 rounds of Gamunex IVIG (2 gm/kg per cycle) were conducted in 2020 without significant relief. The patient also failed to respond to three rounds of rituximab therapy (1000 cc over 6 hours) that took place in the months of January, February and September of 2022. The patient was then tried on Dupilumab therapy and after a 600mg dose at day 0 and a 300 mg dose delivered two weeks later, there was a clinically significant improvement with no new flares. The patient remains on concomitant oral doxycycline (100 mg) once daily and topical 0.1% triamcinolone as needed. The patient denies any adverse drug reactions with the new addition of Dupilumab and is continuing therapy with biweekly 300 mg injections.
Discussion: Bullous Pemphigoid is an autoimmune blistering disease with variable responses to standard therapies. Our case and the literature have shown promising therapeutic results with the use of Dupilumab in treatment resistant BP.
Embargo Period
7-5-2023
Included in
Treatment of bullous pemphigoid with dupilumab: A case report
Philadelphia, PA
Introduction: Bullous Pemphigoid (BP) is an autoimmune blistering condition that results from formation of autoantibodies against basement membrane hemidesmosome proteins, specifically BP180 and BP230. Classic BP presents as pruritic, tense blisters on the trunk and extremities, most commonly in the elderly. BP’s pathophysiology involves IL4/IL5/IL13 and eosinophils. Dupilumab is an IgG monoclonal antibody targeted at the IL4-α receptor that works to attenuate the IL4/IL13 pathway involved in T2 differentiation. Dupilumab has clinical applications for T2 mediated disease and shows promise in the therapy of BP.
Case Description: We report a case of an 83-year-old Asian male who presented in the spring of 2019 with a chief complaint of blistering lesions accompanied by significant pruritus. Medical history was positive for hypertension controlled on amlodipine and losartan and hypercholesterolemia not on any medical therapy. Past medical history and family history were negative for any autoimmune or skin disorders. Tense bullae were noted in the arm, hands, thighs, feet, groin and the head. The blistering rash appeared to be urticarial lesions which were unresponsive to prednisone therapy. Hypersensitivity reaction and drug eruption were suspected.
Initial biopsy revealed spongiotic dermatitis with eosinophilic spongiosis and there was suggestion of BP. A diagnosis of BP was later confirmed on a second biopsy via direct immunofluorescence.
Initial treatment with oral prednisone and doxycycline 100mg bid, topical 0.1% triamcinolone bid and topical 1% silver sulfadiazine was unsuccessful. Every time prednisone was tapered the patient would have flares of new blisters. Sarna was used for managing pruritus. Mycophenolate mofetil (1g BID) and dapsone (25 mg BID and eventually 2x25mg tablets, BID) were attempted but gradually discontinued because of significant neuropathy and paresthesias. 5 rounds of Gamunex IVIG (2 gm/kg per cycle) were conducted in 2020 without significant relief. The patient also failed to respond to three rounds of rituximab therapy (1000 cc over 6 hours) that took place in the months of January, February and September of 2022. The patient was then tried on Dupilumab therapy and after a 600mg dose at day 0 and a 300 mg dose delivered two weeks later, there was a clinically significant improvement with no new flares. The patient remains on concomitant oral doxycycline (100 mg) once daily and topical 0.1% triamcinolone as needed. The patient denies any adverse drug reactions with the new addition of Dupilumab and is continuing therapy with biweekly 300 mg injections.
Discussion: Bullous Pemphigoid is an autoimmune blistering disease with variable responses to standard therapies. Our case and the literature have shown promising therapeutic results with the use of Dupilumab in treatment resistant BP.