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

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.

Dermatoscope of primary localized cutaneous nodular amyloidosis on hallux nail bed

“… 4 Sonagara et al 5 postulated that the yellowish-orange background may be correlated with deposits of amorphous material within the deep dermis and subcutaneous tissue. Similar conclusions were made by Atzori et al, 6 explaining the yellow-orange roundish structures described in their case reports. This is most likely the reason for the orange-pink background in our case ( Fig 2 ), as confirmed by the presence of amorphous eosinophilic deposition in the stroma and within vessel walls ( Figs 3 and 4 ).…”

“… 7 We postulate that this is the same reason we see shiny white lines in our case ( Fig 2 ). Some authors 4 , 6 described hemorrhagic changes and telangiectasias, but these findings were not present in our patient.…”

“…7 We postulate that this is the same reason we see shiny white lines in our case (Fig 2). Some authors 4,6 described hemorrhagic changes and telangiectasias, but these findings were not present in our patient. Several treatment options have been reported for PLCNA, but there is no consensus on the most effective treatment option.…”

Nail Amyloidoma: Two Case Reports of a New Entity

“…Histopathological features of the nodules of both of our patients were also similar to those observed in cutaneous amyloidomas. Histopathology of cutaneous amyloidoma shows a nodular deposit of eosinophilic material in the dermis and/or subcutaneous tissue staining with Congo red and showing apple-green birefringence under polarized light and corresponds to the amyloid deposits [16,17]. Tissue architecture is usually preserved [18], but it can also be replaced by the tumoral deposits of amyloid [16].…”

“…Exclusion of systemic amyloidosis, multiple myeloma, and other light chain-related diseases is mandatory [3,19]. There is usually no systemic involvement in cutaneous AL amyloidoma, which remains a form of localized amyloidosis with an indolent course [17,19]. Nonetheless, a minority of patients [4-15%] with AL amyloidoma may progress to systemic amyloidosis or other hematological dyscrasias [12,17].…”