Author/publish year Study design Number of patients Gender Age (years)/median (range) Duration of BP (months)/median (range) Patients’ comorbidities Previous treatment Concomitant treatment Resolution outcomes Recurrence Adverse events Velin et al. [7 ] (2022) A monocentric real-life study 8 4M, 4F 79.5 (70–87) 5.5 (1–29) Chronic anemia (N = 1); onset of hepatic fibrosis (N = 1); Asthenia (N = 1) Superpotent TCs (N = 8); MTX (N = 3); oral steroids (N = 1); OMZ (N = 2) Oral steroids (N = 8); superpotent topical steroids (N = 5) Nontreatment-related deaths (N = 2); complete response (N = 3); partial response (N = 1); stop for side-effect (N = 1); No remission (N = 1) NR Poor cutaneous tolerance with “buming” sensation (N = 1) Li W et al. [8 ] (2022) Case report 1 M 89 NR Atopic dermatitis; asthma; ulcerative colitis OMZ None Resolution of eczema-like skin lesions and all blisters within 6 weeks Remained symptom-free at 6 months NR Baffa et al. [9 ] (2023) Case report 1 F 91 NR Hypertensive chronic kidney failure; Oral prednisone at a starting dose of 0.5 mg/kg/day and topical clobetasol propionate 0.05% ointment once daily; MPS (3 mg/kg/day) for 3 consecutive days; AZA 50 mg/day; RTX 1000 mg 2 weeks NR Complete resolution in pruritus and blisters within 2 months NR NR Bur et al. [10 ] (2022) Retrospective study 8 M 72 (58–87) NR Melanoma (N = 7); anaplastic thyroid carcinoma (N = 1); oropharyngeal SCC (N = 2); cutaneous SCC (N = 1); Merkel cell carcinoma (N = 1); non-small-cell lung cancer (N = 2); renal cell carcinoma (N = 3); prostate cancer (N = 1) urothelial carcinoma (N = 2) None (N = 4); Doxycycline (N = 3); MTX (N = 1); OMZ (N = 1) TCs (N = 8) Disease remission (N = 8) NR NR Zhang et al. [11 ] (2023) Retrospective study 7 3M, 4F 74 (63–88) 6 (1–36) Hypertension (N = 4); benign prostatic hyperplasia (N = 2); diabetes (N = 2); osteoporosis (N = 1); after macular surgery (N = 1); cirrhosis (N = 1); myelodysplastic syndrome (N = 1); hyperuricemia (N = 1); inguinal hernia (N = 1); pulmonary nodule (N = 1) None (N = 5) tofacitinib (N = 1) OMZ (N = 1) glucocorticoid (N = 1) CsA (N = 1) None (N = 1); prednisolone (N = 1); MPS (N = 5) Significant improvement within 8 weeks (N = 7) No recurrence for 9 months (range:6–12) (N = 6) one patient relapsed at 28 weeks but the condition improved after being given again for q2w (N = 1) A moderate pleural effusion due to nocardia infection (N = 1) Zhou et al. [12 ] (2022) Case report 1 F 17 2 None Intravenous methylprednisolone; oral prednisone; IVIG; RTX; plasmapheresis Oral prednisone; IVIG; RTX; plasmapheresis Pruritus improvement and absence of new lesion within 2 weeks Complete blister resolution and undetectable BP-180 levels at a 4-month follow-up NR Seyed Jafari et al. [13 ] (2021) Case report 1 M 70 24 Obesity; type 2 diabetes mellitus; arterial hypertension TCs; dapsone; MTX; MMF; OMZ TCs; MMF; OMZ Complete remission (3 months) Clinical remission (7-month-follow-up visit) No adverse events Abdat et al. [14 ] (2020) Case series 13 8M, 5F 78 (53–91) 18 (1–60) Hepatitis B core antibody (+) (N = 1) None (N = 1); prednisone (N = 8); IVIG (N = 3); prednisolone (N = 1); MTX, (N = 4); doxycycline (N = 4); mycophenolate (N = 2) RTX (N = 2); niacinamide (N = 3) AZA (N = 1) None (N = 6); MTX (N = 3); prednisone (N = 3); topical steroids (N = 1) Complete remission (N = 7); partial response (N = 4), No remission (N = 2) NR No adverse events Seidman et al. [15 ] (2019) Case report 1 M 89 NR Type 2 diabetes mellitus; thrush; corticosteroid myopathy Doxycycline 100 mg twice daily; nicotinamide 500 mg twice daily; MMF 1000 mg twice daily (peak of 1500 mg twice daily); prednisone 10 mg daily; TCs; OMZ; various antihistamines Prednisone 2.5 mg daily; MMF 500 mg twice daily; doxycycline 100 mg twice daily; nicotinamide 500 mg twice daily; topical clobetasol 0.05% cream; metformin Itching improved within 2 weeks and complete resolution within 7 weeks No recurrence for one year NR Lai et al. [19 ] (2022) Case report 1 M 86 72 Type 2 diabetes mellitus; chronic kidney disease; triple‑vessel coronary artery disease AZA 50 mg daily Systemic steroid treatment (MPS 4–32 mg/day); doxycycline (100 mg twice daily); topical clobetasol 0.05 ointment; systemic antihistamines Complete resolution within 2 months Complete resolution of the pruritus and heremained free of blisters and prurigo nodularis for 10 months NR Shan et al. [20 ] (2022) Case report 1 M 32 18 Pulmonary tuberculosis Oral corticosteroid Prednisolone; isoniazid; rifampicin; ethambutol Itching improved within 1 week; blisters resolved within 2 weeks; disease clearance (12 times injection) NR NR Takamura et al. [21 ] (2022) Case report 1 F 72 1.5 Type 2 diabetes mellitus Minocycline; nicotinic acid amide Dipeptidyl peptidase-4 inhibitors Completely improvement of pruritus within 2 weeks and the skin blisters within 4 weeks No recurrence at least 12 months NR Pop et al. [22 ] (2022) Case report 1 F 59 NR Cervical cancer MPS (1 mg/kg/day divided twice daily); oral prednisone; oral doxycycline 100 mg twice daily; oral niacinamide 500 mg twice daily; oral dapsone 75 mg daily; topical bethamethasone diproprionate 0.05% ointment; triamcinolone 0.1% ointment Oral doxycycline 100 mg twice daily; a prednisone course of 60 mg/day tapered down to 10 mg/day over 4 weeks Disease clearance Remained clear for an additional 6 months NR Bruni et al. [23 ] (2022) Case report 1 M 76 18 Scalp nodular melanoma Doxycycline 100 mg twice a day; MPS 40 mg/day for 10 days; TCs MPS Clinical remission within 6 months NR NR Savoldy et al. [24 ] (2022) Case report 1 M 78 NR Type 2 diabetes mellitus; neurocognitive disease Betamethasone dipropionate and triamcinolone 0.1% ointment; topical triamcinolone and lidocaine Levemir; humalog; glimepiride; hydralazine; hydrocodone; terazosin; clonidine; carvedilol; ezetimibe; allopurinol; vitamin D; atorvastatin; Disease improvement within 6 weeks NR No adverse events Yang et al. [25 ] (2022) Retrospective study 20 10M 10F 72 (54–86) 5 (3–12) Interstitial lung disease (N = 5); tumor (N = 1); neurologic disorder (N = 5); Cchronic renal insufficiency (N = 3); cardiovascular disease (N = 3); diabetes mellitus (N = 4); hypertension (N = 6) Systemic therapy (N = 3) none (N = 17) Low-dose MPS (N = 20) Complete remission (N = 20) NR Eosinophilia (N = 4); herpes zoster (N = 1); deep venous thrombosis or pulmonary embolism (N = 2); gastritis (N = 1) pneumonia (N = 1) Wang et al. [26 ] (2022) Case report 2 M 80 (72–88) 6.5 (1–12) Type 2 diabetes (N = 1) tuberculosis (N = 1) MPS (N = 2); MTX (N = 1) MPS (N = 2) Disease improvement within 2 weeks (N = 2) NR NR Xu et al. [27 ] (2023) Case report 2 1M,1F 65.5 (53–78) 9 (6–12) NR Prednisone (N = 2); ebastine (N = 1); chlorphenamine maleate (N = 1); TCs (N = 1) Prednisone (N = 2) Significant improvement at first treatment and clinical remission at the 8-month (N = 1) significant improvement within 2 weeks (N = 1) No recurrence during the 4-month follow-up (N = 1) NRn(N = 1) No side effects of dupilumab during the 4-month follow-up (N = 1) Saleh et al. [28 ] (2021) Case report 1 M 80 3 NR Topical triamcinolone ointment; prednisone 40 mg daily; doxycycline 100 mg twice daily; niacinamide 500 mg three times per day; MMF 1000 mg twice NR Marked improvement after 2 weeks, followed by complete clearance NR NR Zhang et al. [29 ] (2021) Case report 1 F 61 60 NR MPS (0.5 mg/kg/day) taper; AZA 100 mg daily MPS; topical steroids (clobetasol propionate 0.05% ointment twice/day); AZA Resolution of pruritus and skin blister within one month Clinical remission at the 5-month follow-up visit NR Klepper et al. [30 ] (2021) Case report 1 F 79 NR Melanoma Triamcinolone 0.1% ointment; clobetasol 0.05% ointment; 100 mg of doxycycline twice daily; 180 mg of fexofenadine daily; oral dapsone; emollients; prednisone NR Complete remission of pruritus and BP lesion within 4 weeks NR NR Zhang et al. [31 ] (2021) Retrospective study 8 3M, 5F 64.5 (45.5-71.7 5) 2 (1.25– 49.5) Cardiovascular disease (N = 3); cancers (N = 2); neurologic disorders (N = 1); hyperlipidemia (N = 3) NR MPS (N = 8) (0.6 mg/kg/day) AZA(N = 8) (2 mg/kg/day) Complete remission (N = 5) partial remission (N = 1) no remission (N = 2) One patient relapsed within 32 weeks Osteoporosis (N = 1) Liu et al. [32 ] (2021) Case series 3 1M, 2F 54 (50–68) 17 (3–36) Psychiatric disorder (N = 1); HBV + (N = 2); hypertension (N = 1); type 2 diabetes mellitus (N = 1); arrhythmias with sustained atrial fibrillation (N = 1); stroke (N = 1) MPS 80 mg/d (N = 2); prednisone (N = 1); CsA (N = 1); CTX (N = 2); IVIG (N = 2); dexamethasone (N = 1); MTX (N = 1) MPS (N = 2); MTX (N = 1); CsA (N = 1); IVIG (N = 1); CTX (N = 1) Disease clearance within 1 month (N = 1); disease improvement within 1 week (N = 1) pruritus improved but not the skin lesions (after three injections, N = 1) NR No adverse events Singh et al. [33 ] (2020) Case report 1 F 83 2 Chronic lymphocytic lymphoma; severe congestive heart failure; Alzheimer’s dementia; latent tuberculosis Prednisone NR Itch improved and no new bullae appeared within 3 weeks; complete resolution of disease within 15 weeks NR NR Kaye et al. [34 ] (2018) Case report 1 M 80 s 1.5 Mycobacterium tuberculosis and hepatitis B core antibody (+) Prednisone and immunomodulatory NR Itching improved within 1 week; resolution of all blisters after 3 months No recurrence at least 10 months NR Liang et al. [35 ] (2023) Retrospective study 9 7M, 2F 68 (42–89) 6 (1.5–144) Mycobacterium tuberculosis (N = 5); obsolete pulmonary tuberculosis (N = 2); asthma (N = 1); thyroid disorders (N = 5); sleep disorders (N = 1); hypertension (N = 6); cardiovascular disease (N = 4) type 2 diabetes mellitus (N = 2); old myocardial infarction (N = 1); hepatitis B core antibody (+) (N = 1); osteoporosis (N = 3); COPD (N = 1); ypoproteinemia (N = 1) bronchial stenosis (N = 1); scoliosis (N = 1); hernia cholelithiasis (N = 1); cholecystitis (N = 1); hydrocele (N = 1); skin infection (N = 2); arthritis (N = 1); elevated PSA levels (N = 1); old silent lacunar infarcts (N = 1); diabetic nephropathy (N = 1); old cerebral infarction (N = 1); chronic anemia (N = 1); Alzheimer’s disease (N = 1); chronic kidney disease (N = 1) epilepsy (N = 1) TCs (N = 9) systemic steroids (N = 5) CsA (N = 1) Tripterygium glycosides tablets (N = 3); minocycline (N = 4); thalidomide (N = 1); IVIG (N = 1); CTX (N = 1) TCs (N = 9); systemic steroids (N = 8); CsA (N = 1); Tripterygium glycosides tablets (N = 7); minocycline (N = 8); thalidomide (N = 1); CTX (N = 1) Complete remission (N = 6); partial response (N = 1); lesions improved within 4 weeks discontinued due to cost (N = 2) NR No significant adverse effects
