Primary Localized Cutaneous Nodular Amyloidosis and Limited Cutaneous Systemic Sclerosis: Additional Cases with Dermatoscopic and Histopathological Correlation of Amyloid Deposition

Laura Atzori,Caterina Matucci-Cerinic,Caterina Ferreli,Luca Pilloni,Franco Rongioletti

doi:10.3390/dermatopathology8030028

Abstract

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition due to the plasma cell proliferation and skin deposition of immunoglobulin light chains, without systemic amyloidosis or hematological dyscrasias. The association with autoimmune connective tissue diseases has been reported, especially with Sjogren’s syndrome, and in a few cases with systemic sclerosis. Herein, we describe three cases of PLCNA occurring in women with a diagnosis of limited cutaneous systemic sclerosis and review the literature on the topic to highlight a stereotypical presentation. Moreover, we support the usefulness of dermoscopy, characterized by a yellow–orange waxy pattern surrounded by telangiectasias, for a rapid and non-invasive diagnostic assessment. Thus, when asymptomatic nodules occur on lower limbs of women affected with limited systemic sclerosis, and dermoscopy identifies yellow–orange blotches, a diagnosis of PLCNA can be considered and further confirmed by histopathology. Monitoring for systemic amyloidosis development is advisable, although the risk of progression is considered very low.

Highlights

Primary localized cutaneous amyloidosis occurs when the amyloid is deposited in the skin in the absence of systemic involvement, due to the extracellular accumulation of abnormally folded proteins arranged in the beta-pleated sheet
In primary cutaneous amyloidosis, such as keratin-type macular and lichen amyloidosis, dermoscopy has been reported as a useful diagnostic tool, showing a white or brown central hub surrounded by reticular pigmentation, which corresponds to the histopathological findings of basal hyper pigmentation, amyloid deposition in papillary dermis and pigment incontinence [19]
We have described the diagnostic contribution of dermoscopy in detecting primary localized cutaneous nodular amyloidosis (PLCNA) made by amyloid L [20], presenting with a different pattern characterized by yellow–orange roundish structures, furrowed by whitish hyperkeratotic strikes on an erythematous violaceous background, with more or less elongated telangiectasias [19]

Summary

Introduction

Primary localized cutaneous amyloidosis occurs when the amyloid is deposited in the skin in the absence of systemic involvement, due to the extracellular accumulation of abnormally folded proteins arranged in the beta-pleated sheet. In lichen and macular amyloidosis, amyloid is made by keratin while PLCNA is characterized by an abnormal cutaneous deposition of immunoglobulins, predominantly amyloid light-chain amyloidosis (AL), secondary to a localized proliferation of plasma cells [1,2,3,4,5]. Despite monoclonal plasma cell proliferation, no systemic hematological involvement is usually found at PLCNA diagnosis [2], a 7% risk of myeloma development has been reported [6,7]. The occurrence of PLCNA in autoimmune diseases is well documented, especially in association with Sjögren’s syndrome [8,9,10], where nodular skin amyloidosis can develop inside the breasts and lungs. Our interest is focused on the peculiar dermoscopic characteristics that may help suspecting a diagnosis of PLCNA [18,19]

Results

Discussion

Conclusion