R Laufer Britva1, K T Amber2, A D Cohen3, K Kridin1. 1. Department of Dermatology, Rambam Health Care Campus, Haifa, Israel. 2. Department of Dermatology, University of Illinois at Chicago, Chicago, IL, USA. 3. Siaal Research Center for Family Medicine and Primary Care, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.
Abstract
BACKGROUND: Current treatment paradigms in anti-p200 pemphigoid rely on low levels of evidence, primarily originating from case reports and case series. OBJECTIVE: To systematically review the utilized treatment modalities for anti-p200 pemphigoid and to synthesize the available clinical outcomes of treated patients. METHODS: We conducted a systematic review of the literature using Ovid-Medline (1946-2018), Embase (1947-2018) and Web of Science (1900-2018) databases with a broad and inclusive search strategy along with a subsequent search of retrieved articles. All case reports and case series of patients with anti-p200 pemphigoid were included. RESULTS: Sixty-eight eligible studies comprising 113 anti-p200 pemphigoid patients with a mean age of 65.5 years were included in the qualitative synthesis. The clinical outcome of patients following treatment was reported for 91 (80.5%) patients, of whom 83 (91.2%) had achieved complete remission at least once. Complete remission on-therapy was observed in 51 (56.0%) and complete remission off-therapy in 12 (13.2%) patients. Thirty-six (39.6%) patients had experienced at least one flare during the duration of follow-up. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the leading therapeutic approach (63.0%) required for disease control. Systemic and topical corticosteroids as monotherapy were sufficient to control the disease in 19.6% and 13.0% of cases, respectively. Dapsone was the most commonly used (41.3%) adjuvant agent. The highest rates of complete remission were achieved in patients managed by systemic corticosteroids as monotherapy (100%) and in those managed by systemic corticosteroids with adjuvant agents (90.7%). Conversely, 45.5% of patients treated only by topical corticosteroids experienced at least one relapse during follow-up. CONCLUSION: The vast majority of patients had reached a complete remission during the course of the disease, whereas a considerable proportion of patients experienced at least one relapse. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the most frequently utilized therapeutic approach.
BACKGROUND: Current treatment paradigms in anti-p200 pemphigoid rely on low levels of evidence, primarily originating from case reports and case series. OBJECTIVE: To systematically review the utilized treatment modalities for anti-p200 pemphigoid and to synthesize the available clinical outcomes of treated patients. METHODS: We conducted a systematic review of the literature using Ovid-Medline (1946-2018), Embase (1947-2018) and Web of Science (1900-2018) databases with a broad and inclusive search strategy along with a subsequent search of retrieved articles. All case reports and case series of patients with anti-p200 pemphigoid were included. RESULTS: Sixty-eight eligible studies comprising 113 anti-p200 pemphigoid patients with a mean age of 65.5 years were included in the qualitative synthesis. The clinical outcome of patients following treatment was reported for 91 (80.5%) patients, of whom 83 (91.2%) had achieved complete remission at least once. Complete remission on-therapy was observed in 51 (56.0%) and complete remission off-therapy in 12 (13.2%) patients. Thirty-six (39.6%) patients had experienced at least one flare during the duration of follow-up. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the leading therapeutic approach (63.0%) required for disease control. Systemic and topical corticosteroids as monotherapy were sufficient to control the disease in 19.6% and 13.0% of cases, respectively. Dapsone was the most commonly used (41.3%) adjuvant agent. The highest rates of complete remission were achieved in patients managed by systemic corticosteroids as monotherapy (100%) and in those managed by systemic corticosteroids with adjuvant agents (90.7%). Conversely, 45.5% of patients treated only by topical corticosteroids experienced at least one relapse during follow-up. CONCLUSION: The vast majority of patients had reached a complete remission during the course of the disease, whereas a considerable proportion of patients experienced at least one relapse. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the most frequently utilized therapeutic approach.