Treatment and clinical outcomes in anti‐p200 pemphigoid: a systematic review

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.

Anti-p200 pemphigoid mimicking erythema multiforme

“…Dapsone was the leading adjuvant agent used. 9 In conclusion, the present case appearing with EM-like lesions confirms the heterogeneous clinical profile of anti-p200 pemphigoid. Dermatologists should be familiar with this for a timely diagnosis.…”

Skin Blister Formation and Subepidermal Bullous Disorders

“…A combination of systemic corticosteroids and adjuvant immunomodulatory agents is preferred for systemic treatment. Unlike bullous pemphigoid, dapsone is the most preferred adjuvant agent in this disease [69].…”

Penfigoide anti-p200: ¿enfermedad poco frecuente o subdiagnosticada?

“…descubrieron que el 90% de estos autoanticuerpos reconocían como antígeno a la laminina gamma-1 detectada por ELISA y acuñaron el término penfigoide antilaminina gamma-1 3,6,7 (Gráfico 1). Sin embargo, los estudios posteriores in vivo y ex vivo no hallaron evidencia clara del papel patogénico de estos anticuerpos y concluyeron que se necesitaban más estudios para esclarecer la fisiopatogenia de esta entidad [7][8][9] .…”

“…Afecta principalmente a los asiáticos, con una media de edad de aparición de 65 años. Suelen ser más jóvenes que los pacientes con PA 2,6,7 .…”

“…En un artículo de revisión realizado en Israel y Estados Unidos, que incluyó a 92 pacientes con esta entidad, se estableció que el mejor esquema terapéutico es la combinación de corticosteroides sistémicos en dosis de 0,8-1 mg/kg/día y dapsona. Sin embargo, también se describieron pacientes tratados, con buenos resultados, con otros fármacos que inhiben la migración de polimorfonucleares (como minociclina o colchinina) o inmunosupresores 7 .…”