Relapse of bullous pemphigoid: an update on this stubborn clinical problem

Abstract

Bullous pemphigoid (BP) is a chronic autoimmune blistering disorder that has a predilection for the elderly. It is characterized by a poor prognosis due to its high mortality rate and the tendency to relapse. The relapse rate of BP ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission. Clinical interventions aimed to prevent early relapses could reduce potential complications from first-line treatment and make follow-up care easier for clinicians in practice, thus improving the prognosis of BP and the quality of patients' lives. In this article, we attempt to review previous studies from the medical literature concerning relapse and risk factors related to relapse in BP patients. Treatments found to be able to reduce or increase relapse rate were also discussed. We propose that clinicians be aware of these risk factors and manage proper treatment accordingly. Key Messages • The relapse rate of bullous pemphigoid ranges from 27.87% to 53% after disease remission, while the majority of relapses occur early (within 6 months) during remission. • Ageing, disease severity, neurological disorder, serum ECP, BAFF, IL-17, IL-23, CXCL10 and anti-BP180 antibody levels have been shown to be related to relapse, while confirmation studies are needed before using them for guidance for relapse prevention. • Longer usage of corticosteroid with a low dose, combination of immunosuppressants and intravenous immunoglobulin are helpful in lowering relapse rate.

IL-13 Genetic Susceptibility to Bullous Pemphigoid: A Potential Target for Treatment and a Prognostic Marker

“…Our analysis of the correlation between IL-13 and recurrence found that both the rs20541 and rs1800925 genotypes significantly correlate with recurrence, suggesting that patients with some genotypes be genetically more susceptible. Relapse in BP remained a persistent issue for patients and clinicians and has contributed substantially to the morbidity and mortality from BP ( 36 ). Because high IL-13 expression and gene polymorphisms are associated with BP and clinical relapse of BP, we hypothesize that IL-13 gene polymorphisms have an effect on IL-13 gene expression.…”

“…BP patients were followed up by telephone calls or in our outpatient office for prognostic information. The definition of relapse is the appearance of at least three new lesions in one month (including blisters, urticarial plaques, or eczematous lesions) or no less than one large (>10 cm in diameter) urticarial plaque or eczematous lesion that does not heal within one week, or the extension of original lesions or daily pruritus in the adequately managed patients within disease control ( 36 ).…”

Risk Factors for Mucosal Involvement in Bullous Pemphigoid and the Possible Mechanism: A Review

“…One finding revealed a significant association between dermal eosinophilia and severity of BP ( 46 ). An elevated eosinophil count is thought to increase the risk of relapse and is a predictor of poor prognosis ( 47 ). Thus, eosinophils may be a putative therapeutic target for BP.…”

Penfigoide ampolloso

“…El penfigoide ampolloso (PA) se caracteriza por episodios espontáneos de exacerbaciones y remisiones (meses o años); 30% de los pacientes recae dentro del primer año (aquellos con afección extensa y factores de riesgo como demencia), y 50% reincidirá a los tres meses posterior a la suspensión del tratamiento. 14 El pronóstico se ve afectado por la edad y el estado funcional. La mortalidad en adultos mayores de países occidentales durante el primer año se calcula en 10-40%.…”