Primary localised cutaneous amyloidosis--a systematic review

Abstract

Introduction: Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the literature and to evaluate the risk of developing systemic amyloidosis (SA) and the risk of local recurrence of primary localised cutaneous amyloidosis (PLCA). The method of treatment was compared to the risk of local recurrence.

Methods: A literature search produced 77 articles with localised cutaneous amyloidosis, 23 articles were excluded; thus, a total of 54 articles were included.

Results: A total of 94 patients were included with a male:female ratio of 1.2:1.0. The median age was 57 years (range 24-87 years). The most common tumour localisation was in the head and neck region with a total of 38 lesions (34%), and 20 patients (22%) had two or more lesions in different locations. The nodular subtype was reported in 65 patients (69%). Only 29 patients received therapy with eight patients having two or more treatments (28%). Eight patients (9%) had local recurrence and all were nodular PLCA, which were mainly seen in males and localised in the face. One patient developed SA (1%); in fact, this was the only patient who was positive for monoclonal amyloid light chain amyloidosis by immunoelectrophoresis of the serum.

Conclusion: Our review suggests that PLCA is a benign disease that has a good prognosis and that it is associated with a low risk of developing SA (1%). The risk of developing local recurrence or developing new lesions was 9%, and no significant differences were found when compared to the primary treatment.