A systematic review and meta-analysis of treatments and outcomes of primary localized cutaneous amyloidosis.

Abstract

Background: Primary localized cutaneous amyloidosis (PLCA) is a skin-limited disorder characterized histologically by amyloid deposition in the papillary dermis. This study aimed to review current treatment strategies and provide an updated perspective on the treatment of PLCA.

Methods: We searched the PubMed, EMBASE and Cochrane Library databases for eligible studies. Cohort studies were meta-analyzed using a random effects model to evaluate the outcomes of different treatments, while case reports and case series were evaluated using the Mann-Whitney U test.

Results: Overall, 117 studies (535 patients) were included. Surgery favoured nodular amyloidosis (NA), with statistically significant differences for the other therapies. For the non-NA group, 62 case-level studies (79 patients) and 20 cohort studies (418 patients) were analyzed separately. Although there were no statistically significant differences between therapies in the case-level studies, biological agents and JAK inhibitors may be a promising treatment for refractory lesions. Of the cohort studies, the partial response (PR) rate was 100.0%, 100.0%, 97.5%, 96.9%, and 94.4% of transcutaneous electrical nerve stimulation, micro-needling, laser therapies, topical therapies, and systemic immunosuppressants; the complete response (CR) rate was 22.2% and 2.5% of surgical interventions and laser therapies.

Conclusion: This study suggest that surgery is the most effective option for NA and laser therapy is recommended for non-NA patients. Biologic agents and JAK inhibitors may be promising treatment options for lesions that do not respond to conventional therapies.