Results
itching, bullae, hypertension, erythema, herpes, reduction in skin lesion, eosinophilic leukemia, parasite infection, eosinophilia, pruritus, hyperkalemia, blisters, herpes zoster, coronary heart disease, skin lesions, allergic diseases
VIRUS, OEDEMA OF THE LOWER LIMBS, RECURRENCE, HYPERTENSION, DISEASE, ERYTHEMA, HERPES, KIDNEY INSUFFICIENCY, EOSINOPHILIC LEUKEMIA, EOSINOPHILIA, CARDIAC INSUFFICIENCY, ERUPTIONS, REMISSION, ADVERSE EVENT, HERPES ZOSTER, CORONARY HEART DISEASE, SKIN LESIONS

Nine patients (90%) receiving dupilumab therapy showed notable improvements in their pruritus symptoms and BP eruptions. Seven patients (70% of the total) obtained CR. On average, it took 8.3 weeks (58 days) for seven patients to achieve CR. In our case series, 100% of mild-to-moderate cases attained CR. Of the severe BP patients (cases 1, 6, 7, 8, 9, and 10 in which BPDAI exceeded 57) (In case 1, the patient initially received three courses of a higher dose of 600 mg SC every 2 weeks, followed by 300 mg SC every 2 weeks. In the interim, 40 mg of TwHF was also administered. The fact that the daily dose of methylprednisolone in case 1 was 16 mg, which is far below the recommended prescribing regimen of 0.75–1 mg/kg for severe patients (Clinical presentation before and after therapy in case 1. Erythematous patches and tense bullae appear on the trunk, extremities, and hands before treatment A 98-year-old male patient with intolerable pruritus for at least 1 month presented to our department in case 6. He suffered from hypertension and coronary heart disease in an inferior general condition. BP symptoms improved after treatment with topical corticosteroids, minocycline, and niacinamide for 3 weeks. However, he swiftly developed new blisters and experienced intractable itching. Owing to contraindications with oral corticosteroids and extremely old age, dupilumab monotherapy was introduced. Prior to dupilumab administration, the eosinophil count and anti-BP180 antibody concentration were 5.9% and 16 U/ml, respectively. However, there were no laboratory results after treatment because he was unable to visit the hospital during the COVID-19 pandemic. We made contact via the PUMCH online communication system during the follow-up period. Two days after the first injection, his pruritus significantly improved, and he could fall asleep soundly. After five courses of dupilumab monotherapy, he reported CR and no new lesions. Overall, the patient received a total of seven courses of dupilumab monotherapy and was completely symptom free. During the 1-year follow-up period, there were no reports of disease flare-ups.In case 7, a younger patient initially received 40 mg of methylprednisolone per day, but more than 50 new blisters developed after 3 days of treatment, and he suffered from severe pruritus. The initial loading dose of 600 mg of dupilumab by injection was prescribed. He reported a significant improvement in previously identified skin lesions and pruritus symptoms the next day. Following two injections of dupilumab, he was nearly free of blisters and had smaller erythematous regions. Additionally, a substantial decrease in eosinophil counts was observed (from 20% before therapy to 0.1% after the first course of dupilumab). The patient reported no new blister formation and complete symptom alleviation after three injections of dupilumab. Anti-BP180 antibody titers decreased from over 150 U/L to 113 U/L after 12 weeks. Additionally, dupilumab markedly facilitated methylprednisolone tapering (from an initial dose of 40 mg/day to 10 mg/day in 6 months).Case 8 patient reported a reduction in the edema of the lower limbs and feet after the first injection of dupilumab. However, she continued to develop new blisters on the right upper extremity. The percentage of eosinophils significantly increased from 38.8% before treatment to 65.2% and 67.7% 1 and 2 weeks after the first injection, respectively. She experienced intense pruritus and impatience. We introduced the immunosuppressant TwHF at a dosage of 40 mg daily after two courses of dupilumab. Additionally, topical corticosteroids were applied to her newly formed blisters. The patient reported a marked reduction in skin lesion counts and pruritus after TwHF introduction. Additionally, eosinophil levels decreased to 31%. However, 2 days after the fourth injection of dupilumab, she described a relapse characterized by the appearance of new erythema on her face. A compound betamethasone injection (containing 7 mg of betamethasone) was prescribed. After 4 days of betamethasone injections, her skin lesions improved significantly, and her eosinophil count returned to normal levels. Until the present time, the patient has reported CR of skin lesions and pruritus with a normal eosinophil count. We did not rule out the roles of TwHF and betamethasone and classified the patient as being in partial remission even though the patient ultimately attained satisfactory results. During the 44 weeks of treatment with dupilumab plus TwHF and topical corticosteroid, we observed a significant decrease in total IgE (from 1,090 IU/ml after three injections to 33.9 IU/ml) and anti-BP180 (from 115 U/ml before dupilumab initiation to 20 U/ml). After 44 weeks of combination treatment, we chose to discontinue dupilumab. At her most recent clinic visit (six months subsequent to CR), she was still maintaining CR with 40 mg of TwHF daily. No recurrence of the disease was reported. It was noteworthy that the patient had herpes zoster 3 months previously, and treatment with dupilumab did not result in a recurrence of the herpes virus.Case 9 showed a modest improvement in pruritus and bullae after two courses of dupilumab. However, the eosinophil count was markedly increased to 38.7%, 37.6%, and 42.4% 3 days, 1 week, and 2 weeks after the first injection, respectively. We had to reinitiate conventional systemic immunosuppressive therapies. Intravenous methylprednisolone (40 mg daily) was administered 1 week after two injections of dupilumab. Four days later, she experienced a perceptible reduction in itching, and no new blisters appeared. We tapered methylprednisolone to 20 mg daily while prescribing 60 mg of TwHF daily. After three administrations of dupilumab, methylprednisolone dosage was reduced by 16 mg daily. However, the patient reported a relapse of BP four days after methylprednisolone tapering. She suffered intolerable pruritus and the rapid development of over 30 new lesions. The daily dose of methylprednisolone was increased to 32 mg so that she ultimately achieved disease control. While receiving anti-IL-4 receptor therapy, cardiac insufficiency, kidney insufficiency, and hyperkalemia did not worsen in this case. This patient was classified as having no improvement because disease control was achieved by increasing the dosage of conventional systemic immunosuppressive therapies. In cases 8 and 9, the eosinophil count was substantially elevated following dupilumab monotherapy. In both patients, parasite infection, allergic diseases, and eosinophilic leukemia were ruled out. We proposed that eosinophilia following the initiation of dupilumab could be an adverse event in both cases.Case 10 was referred to our dermatology department due to resistance to traditional BP treatment. We implemented a systemic combination therapy consisting of 300 mg of dupilumab SC every 2 weeks and 40 mg of TwHF daily. After 2 weeks of combination therapy, the patient reported a persistent increase in the number of bullae (In the case series, four mild-to-moderate patients (BPDAI not exceeding 57) (