Ultraviolet-A1 phototherapy in the treatment of primary diffuse cutaneous amyloidosis: an additional phototherapy regimen for cutaneous amyloidosis with review of treatment options

Abstract

SummaryPrimary cutaneous amyloidosis (PCA) is a chronic pruritic skin disorder of unknown origin. We report on the case of a 48-year-old Indian woman with diffuse biphasic amyloidosis in which UVA1 phototherapy resulted in marked improvement of skin lesions and pruritus. UVA1 phototherapy was administered as monotherapy five times a week for 3 weeks with an exposure at a single dose of 130 J/cm 2 resulting in a cumulative UVA1 dose of 1950 J/cm 2 . After treatment, the skin appeared less pigmented with flattened papules, and the histology revealed the disappearance of amyloid deposits and the presence of melanophages in the dermis. A comprehensive review of the literature on the treatment options for PCA showed no controlled trials available. However, case series indicated phototherapy (UVB, narrow band UVB, photochemotherapy) as the most successful treatment.

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.…”