Biological Significance of the Association Between Cutaneous Al-amyloidoma and Sjögren Syndrome

Saggini, Andrea; Cerroni, Lorenzo; Lora, Viviana; Cota, Carlo

doi:10.1097/dad.0000000000001528

Cited by 3 publications

(9 citation statements)

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“…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].…”

Section: Discussionmentioning

confidence: 99%

“…Tissue architecture is usually preserved [18], but it can also be replaced by the tumoral deposits of amyloid [16]. A sparse perivascular infiltrate consisting of lymphocytes and plasma cells, which are usually monotypic, is also visible in cutaneous AL amyloidoma [3,[17][18][19]. Lesions are indistinguishable from cutaneous nodular deposits in systemic AL amyloidosis [16].…”

Section: Discussionmentioning

confidence: 99%

“…AL amyloidoma is thought to be associated with a localized proliferation of monoclonal plasma cells, which presumably produce the AL [1,17]. It may represent a localized "smoldering" type of extramedullary plasma cell neoplasm or a form of extranodal marginal zone lymphoma [3,19,20]. Exclusion of systemic amyloidosis, multiple myeloma, and other light chain-related diseases is mandatory [3,19].…”

Section: Discussionmentioning

confidence: 99%

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Nail Amyloidoma: Two Case Reports of a New Entity

Bonito¹,

Kolivras²,

Saß³

et al. 2023

Skin Appendage Disord

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Introduction: Amyloidosis is a group of diseases characterized by extracellular deposits of abnormal insoluble proteins in different tissues. Amyloidoma is a localized tumoral deposit of amyloid in the absence of systemic amyloidosis, and it has been described in different anatomic sites. We report two cases of amyloidoma in the nail unit and provide insights into this recently described entity. Case Presentation: Both cases presented as an asymptomatic, slowly growing nodule underneath the distal nail bed of a toe with associated onycholysis. Histopathology was characterized in both patients by the presence of deposits of Congo red-positive, homogeneous, amorphous, and eosinophilic material within the dermis and subcutaneous tissue admixed with aggregates of plasma cells. In both cases, an extensive workup excluded systemic amyloidosis. Treatment was based on local excision, and no local recurrence or progression to systemic amyloidosis was observed at 1 year of follow-up. Conclusion: These are the first reports of amyloidomas of the nail unit. The clinical and histopathological presentations parallel those of an amyloidoma affecting the skin. Local excision seems to be an efficient treatment modality, but long-term follow-up is warranted in order to exclude recurrence, an associated marginal B-cell lymphoma, or progression to systemic amyloid L amyloidosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Nail Amyloidoma: Two Case Reports of a New Entity

Bonito¹,

Kolivras²,

Saß³

et al. 2023

Skin Appendage Disord

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“…In some cases, an association of PLCNA with chronic lymphocytic leukemia and some autoimmune disorders such as Sjogren syndrome, systemic lupus erythematosus, or limited scleroderma, among others, has been described. 2,6 The therapeutic approach of PLCNA is challenging because there is not a clearly effective treatment option, and local recurrence is common. Different treatment modalities have been described, including surgical excision, CO 2 laser, cryotherapy, dermabrasion, electrodesiccation and curettage, and pulsed dye laser.…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic Solitary Nodule in the Interdigital Space: Answer

Quintana-Codina

Fernández‐Figueras

Setó-Torrent

et al. 2021

The American Journal of Dermatopathology

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“…Cutaneous deposits of AL-amyloid without systemic involvement, which have been termed AL-amyloidomas, are considered by some authors to represent an unusual manifestation of primary cutaneous marginal zone lymphoma (pcMZL) when accompanied by a variably dense lymphoplasmacytic infiltrate demonstrating evidence of light-chain restriction. [8][9][10] Herein, we report an additional case of primary cutaneous amyloid elastosis to frame a discussion of possible etiologic associations and highlight the variable clinical presentation and prognosis of this unique histopathologic entity.…”

Section: Introductionmentioning

confidence: 99%

A Second Case of Localized Cutaneous Amyloid Elastosis: Expanding the Discussion of Associations With a Unique Histopathologic Entity

Roberts

Dittmer

Vidal

et al. 2021

The American Journal of Dermatopathology

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Amyloid elastosis is an exceedingly rare form of amyloidosis characterized by amyloid material deposited on dermal elastic fibers. Most reported cases have been associated with systemic amyloid light-chain amyloidosis. A single previously reported case of amyloid elastosis showed evidence that the amyloid material was derived from light-chain proteins and was associated with a monoclonal plasma cell infiltrate but failed to demonstrate systemic involvement. As a result, the case was felt to represent localized cutaneous amyloid elastosis. We present a case of localized cutaneous amyloid elastosis that is not associated with a definitive monotypic plasma cell population or with systemic amyloidosis. We also review the clinical and histopathologic features of reported cases of amyloid elastosis and discuss possible etiologic considerations. Because amyloid elastosis can be either localized to the skin or associated with systemic involvement, additional workup to exclude an underlying plasma cell dyscrasia or hematologic malignancy is warranted.

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Biological Significance of the Association Between Cutaneous Al-amyloidoma and Sjögren Syndrome

Cited by 3 publications

References 10 publications

Nail Amyloidoma: Two Case Reports of a New Entity

Nail Amyloidoma: Two Case Reports of a New Entity

Asymptomatic Solitary Nodule in the Interdigital Space: Answer

A Second Case of Localized Cutaneous Amyloid Elastosis: Expanding the Discussion of Associations With a Unique Histopathologic Entity

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