Primary Localized Cutaneous Nodular Amyloidosis Research Articles

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

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition attributed to plasma cell proliferation and the deposition of immunoglobulin light chains in the skin without association with systemic amyloidosis or hematological dyscrasias. It is not uncommon for patients diagnosed with PLCNA to also suffer from other auto-immune connective tissue diseases, with Sjögren's syndrome (SjS) showing the strongest association. This article provides a literature review and descriptive analysis to better understand the unique relationship between these two entities. To date, 34 patients with PLCNA and SjS have been reported in a total of 26 articles. The co-existence of PLCNA and SjS has been reported, especially in female patients in their seventh decade of life with nodular lesions on the trunk and/or lower extremities. Acral and facial localization, which is a typical localization of PLCNA in the absence of SjS, seems to be much more unusual in patients with associated SjS.

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

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.

Primary Localized Cutaneous Nodular Amyloidosis on a Toe: Clinical Presentation, Histopathology, and Dermoscopy Findings.

Citation: Ferreira ILO, Fernandes EL, Lapins J, Benini T, Silva LC, Lanzoni MA, Steiner D. Primary localized cutaneous nodular amyloidosis on a toe: clinical presentation, histopathology, and dermoscopy findings. Dermatol Pract Concept. 2019;9(3):235-236. DOI: https://doi.org/10.5826/dpc.0903a18

An exceptional location of primary localized cutaneous nodular amyloidosis

An exceptional location of primary localized cutaneous nodular amyloidosis

Primary localized cutaneous nodular amyloidosis

Primary localized cutaneous nodular amyloidosis

Nodular primary localized cutaneous amyloidosis in a 54-year-old man

Nodular primary localized cutaneous amyloidosis in a 54-year-old man

Primary Localized Cutaneous Nodular Amyloidosis of the Cheeks

AbstractWe report the rare case of a primary localized cutaneous nodular amyloidosis (PLCNA), which clinically appeared as waxy, yellow-red infiltrated plaques with telangiectasia on the face of a 52-year-old woman. Diagnosis was confirmed by immunohistochemistry results. Histologically, diffuse amyloid tissue infiltration could be revealed. Amyloid stained positive for lambda, but negative for kappa light chain antibodies whereas no proliferation of clonal plasma cells in the bone marrow was found.

Amylose cutanée nodulaire primitive localisée : un défi diagnostique et thérapeutique

Nodular primary localized cutaneous amyloidosis (PLCA) is a rare subtype of localized cutaneous amyloidosis in which amyloid protein is derived from immunoglobulin light chains. Follow-up for progression to systemic amyloidosis or autoimmune disease is mandatory. No consensus exists regarding treatment. We report a case of nodular PLCA in a 49-year-old man, presenting as an asymptomatic nodule of the nose. Skin biopsy revealed diffuse deposition of amyloid associated with plasmocyte proliferation. Monotypic kappa light-chain restriction was observed. Extensive systemic evaluation, including bone marrow biopsy and PET scan, was negative. Protein electrophoresis and immunofixation in serum and urine were normal. The nodule was treated with radiotherapy but there was no response. Mohs micrographic surgery (MMS) was performed with no recurrence at 6 months of follow-up. No systemic progression was observed one year after the initial diagnosis. Since nodular PLCA may have a cutaneous presentation similar to that of primary systemic amyloidosis, evaluation for systemic amyloidosis is necessary. Treatment of amyloidosis is difficult. Radiotherapy appears ineffective in treating this type of primary cutaneous amyloidosis, and surgical treatment, where possible, is a good option, especially with MMS, which allows both controlled excision and minimal margins.

A case presentation of nodular primary localized cutaneous amyloidosis: Learning points and update

A case presentation of nodular primary localized cutaneous amyloidosis: Learning points and update

Nodular primary localized cutaneous amyloidosis: presentation of 2 case reports and literature review

The nodular primary localized cutaneous amyloidosis is rare and characterized by skin deposition of amyloid substance. There are cases of progression to invasive disease. The type of amyloid substance is not specific to this disease, also being found in primary systemic amyloidosis or systemic amyloidosis associated with multiple myeloma. The authors describe two cases of nodular primary localized cutaneous amyloidosis. A 70-year-old man underwent excision of papule on the mouth left commissure, and a 39-year-old woman with a vulva’s labia majora swelling that was excised, in which the histology revealed a nodular amyloidosis, both without systemic involvement. Although the prognosis is benign, the follow-up of the patient is required for early detection of a possible evolution to systemic disease, or exclusion of an underlying systemic amyloidosis.

Nodular localized primary cutaneous amyloidosis: a bullous variant

Primary cutaneous amyloidosis describes a group of disorders in which amyloid is deposited in the skin without evidence of systemic involvement. Nodular localized primary cutaneous amyloidosis (NLPCA) is a rare form of these skin-restricted amyloidoses. We present an unusual case of NLPCA in a 51-year-old man, who had clinical and histopathological evidence of subepidermal bullous formation, a unique feature in NLPCA. The possible pathogenesis of this change is discussed.

Nodular primary localized cutaneous amyloidosis in a patient with pulmonary sarcoidosis.

Nodular primary localized cutaneous amyloidosis in a patient with pulmonary sarcoidosis.

Primary localized cutaneous nodular amyloidosis that appeared in a patient with severe atopic dermatitis

Primary localized cutaneous nodular amyloidosis that appeared in a patient with severe atopic dermatitis

Nodular amyloidosis in a patient with liver cirrhosis

A 43-year-old Japanese man with alcoholic liver cirrhosis developed a nodule on the face 1 year prior to presentation. Histopathological examination showed amyloid deposition in the entire dermis, with numerous plasma cells. Nodular primary localized cutaneous amyloidosis is a rare form of amyloidosis, which needs long-term follow-up because of the possibility of the development of systemic amyloidosis. Also, this type of cutaneous amyloidosis may have other complications.

Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare subtype of localized cutaneous amyloidosis and can be associated with various connective tissue disorders. It can be difficult to treat and past therapies include surgical excision, dermabrasion, electrodessication and curettage, cryotherapy and laser therapy. We present a case of a middle-aged woman with PLCNA associated with CREST (calcinosis, Raynaud phenomenon, oesophageal motility disorders, sclerodactyly and telangiectasia) syndrome and Sjögren's syndrome responding to cyclophosphamide with no new amyloid deposits and resolution of skin ulceration after many years of resistance to drug therapy. It is important to monitor these patients for progression into systemic amyloidosis.

Nodular localized primary cutaneous amyloidosis and primary marginal zone B‐cell lymphoma on the nose: treatment with microscopically controlled surgery

Nodular localized primary cutaneous amyloidosis and primary marginal zone B‐cell lymphoma on the nose: treatment with microscopically controlled surgery

Primary Localized Cutaneous Nodular Amyloidosis Following Local Trauma

Primary localized cutaneous nodular amyloidosis (nodular amyloidosis) is a rare and distinct type of amyloidosis, in which amyloid L deposition is limited to the skin and typically manifested as a tumefactive nodule on the acral sites. However, the definite cause of nodular amyloidosis is still unknown. Although it is relatively well known that the amyloid deposits in nodular amyloidosis originate from immunoglobulin light chains secreted by local plasma cells, traumatic injury to the skin has rarely been recognized as a triggering factor of nodular amyloidosis. Herein, we present a case of a 50-year-old male patient with primary localized cutaneous nodular amyloidosis, which occurred after local trauma, and discuss the relationship between traumatic damage and dermal amyloid L deposition.

Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome

International Journal of DermatologyVolume 49, Issue 2 p. 229-230 Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome Michael Shiman BS, Michael Shiman BS Miami, FLSearch for more papers by this authorCarlos Ricotti MD, Carlos Ricotti MD Miami, FLSearch for more papers by this authorMaria Miteva MD, Maria Miteva MD Miami, FLSearch for more papers by this authorFrancisco Kerdel MD, Francisco Kerdel MD Miami, FLSearch for more papers by this authorPaolo Romanelli MD, Paolo Romanelli MD Miami, FLSearch for more papers by this author Michael Shiman BS, Michael Shiman BS Miami, FLSearch for more papers by this authorCarlos Ricotti MD, Carlos Ricotti MD Miami, FLSearch for more papers by this authorMaria Miteva MD, Maria Miteva MD Miami, FLSearch for more papers by this authorFrancisco Kerdel MD, Francisco Kerdel MD Miami, FLSearch for more papers by this authorPaolo Romanelli MD, Paolo Romanelli MD Miami, FLSearch for more papers by this author First published: 21 January 2010 https://doi.org/10.1111/j.1365-4632.2008.04058.xCitations: 12Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume49, Issue2February 2010Pages 229-230 RelatedInformation

Primary localized cutaneous nodular amyloidosis: A cutaneous manifestation of Sjögren syndrome

Primary localized cutaneous nodular amyloidosis: A cutaneous manifestation of Sjögren syndrome

Amiloidosis cutánea primaria localizada nodular con patrón diseminado

Amyloid is a proteinaceous material that is deposited in the tissues in a large variety of clinical contexts; in the skin it can be found with or without concomitant systemic disease. Primary localized cutaneous amyloidosis encompasses those amyloidoses restricted to the skin without involvement of other systems. The most common forms within this group are macular and lichen amyloidosis. Nodular amyloidosis is extremely rare, and there are notable differences in clinical presentation, prognosis, histology, and pathogenesis between this entity and the macular and lichenoid variants.We report a new case of nodular primary localized cutaneous amyloidosis with disseminated plaques and nodules in which no systemic disease developed in the 3 years following the appearance of the lesions.