Primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions and an excellent response to systemic acitretin

Abstract

Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory, particularly when the disease is severe and extensive. We describe a rare case of primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions that showed an excellent response to systemic acitretin.

Primary Localized Cutaneous Amyloidosis of Keratinocyte Origin: An Update with Emphasis on Atypical Clinical Variants

“…The literature regarding PLCA treatment is scarce and limited to case series and case reports [8]. Despite the multitude of treatment options, no gold standard has been established as none are curative or uniformly effective and most therapies aim at breaking the itch-scratch cycle (Table 1) [8,26,28,36,37,[56][57][58][59][60][61][62][63][64][65][66][67][68][69][70][71][72]. Mild cases of PLCA respond, to some extent, to potent topical corticosteroids with an added benefit when given under occlusion or combined with a keratolytic agent [56].…”

Persistently itchy papules

“…• intralesional corticosteroid injections • oral acitretin or topical retinoids [16][17][18][19][20] • phototherapy (pulse ultraviolet A, narrowband ultraviolet B) and laser (fractional carbon dioxide laser) 12,13,21 • cryotherapy, cautery and curettage, and dermabrasion. Referral to a dermatologist can also be considered.…”