Three cases of localized cutaneous nodular amyloidosis in patients with limited systemic sclerosis and a brief literature review

Abstract

Localized cutaneous nodular amyloidosis (LCNA) is the rarest form of localized cutaneous amyloidosis. In patients with LCNA, local plasma cells secrete immunoglobulin light chains called amyloid L via an unknown mechanism. LCNA has been associated with autoimmune connective tissue diseases such as most commonly Sjögren syndrome. A few reported cases of LCNA are associated with limited systemic sclerosis (LSSc). We report three cases of LCNA in patients with LSSc to add to the existing literature, discuss the disease association and proposed pathophysiology, and briefly review the existing information in current literature. It is important to closely follow patients with LCNA to monitor progression to systemic amyloidosis.

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

“…The occurrence of cutaneous amyloidosis in lcSSc patients is probably more frequent than reported [ 11 , 12 , 13 , 14 , 15 , 16 , 17 ], with 12 cases of PLCNA including the present cases ( Table 1 ). PLCNA usually follows lcSSc by several years and frequently occurs on the lower limbs of adult women affected by lcSSc.…”

“…None of our PLCNA cases or those retrieved from the literature associated with lcSSc had such an evolution: this detail is important considering the absence of consensus recommendation on PLCNA treatment [ 23 ]. In a few cases, local intralesional triamcinolone has been reported [ 14 ], as well as surgical removal, debulking, dermo abrasion and pulsed dye laser [ 7 , 9 , 12 , 13 , 14 ], with variable results, and frequent recurrence of the nodules after any procedure. Our patients refused the therapy due to the asymptomatic nature of the lesions, the risk of scarring sequelae and the possibility of new lesion occurrence.…”

“…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 also develop inside the breasts and lungs. Herein, we report three cases of PLCNA associated with limited cutaneous systemic sclerosis (lcSSc), with a review of the literature [ 11 , 12 , 13 , 14 , 15 , 16 , 17 ]. In addition to histopathology, our interest is focused on the peculiar dermoscopic characteristics that may help suspecting a diagnosis of PLCNA [ 18 , 19 ].…”

Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome

“…The majority of case reports documenting PLCNA in association with autoimmunity have been in the setting of SjS. PLCNA has also been described in association with other connective tissue diseases such as CREST syndrome (calcinosis, Raynaud’s syndrome, esophageal involvement, sclerodactyly, and telangiectasia) [ 67 , 68 ], systemic sclerosis [ 20 , 69 ], and systemic lupus erythematosus [ 70 ]. This suggests that the dysregulation of plasma cells in the skin may be related to the auto-immune imbalance that is inherent in connective tissue diseases.…”

A 71-year-old Woman with CREST Syndrome and Multiple Waxy Facial Papules and Plaques: A Quiz

“…Upon revisiting the association between PLCNA and CREST syndrome, the reported cases predominantly involved female patients (84%) with a median age of 61.5 years and 80% of the lesions were localized in the lower extremities, presenting a contrast to the facial and acral lesions typically observed in PLCNA cases not associated with CREST syndrome. On average, PLCNA manifested in patients with CREST syndrome after a mean evolution period of 7.9 years; the mean duration of PCLNA until diagnosis was 4.7 years ( 4 – 6 ).…”