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Cureus 2022 Feb 3;14(2):e21870. Epub 2022 Feb 3.

Dermatopathology, Advanced Dermatology and Cosmetic Surgery, Delray Beach, USA.

An 83-year-old Caucasian male presented with a history of asymptomatic yellow-orange macules and plaques concentrated on his trunk and proximal extremities that have been slowly progressing for the past three years. A punch biopsy revealed the presence of eosinophilic amorphous and fissured material within the superficial and interstitial dermis consistent with nodular amyloidosis. With the lack of concurrent systemic symptoms and negative systemic laboratory workup, the patient was diagnosed with disseminated primary localized cutaneous nodular amyloidosis (PLCNA). Read More

Case Rep Dermatol 2021 Sep-Dec;13(3):502-505. Epub 2021 Oct 21.

Clinic of Plastic and Reconstructive Surgery, Marche Polytechnic University Medical School, Ancona, Italy.

Nodular cutaneous amyloidosis represents the rarest variant of primary localized cutaneous amyloidosis. The proposed management ranges from topical or systemic agents to surgical treatment. Complete surgical excision is advisable due to its potential progression to systemic amyloidosis due to dermis and subcutaneous tissue infiltration. Read More

J Investig Med High Impact Case Rep 2021 Jan-Dec;9:23247096211058488

Joan C. Edwards School of Medicine, Marshall University, Huntington, WV, USA.

Nodular localized cutaneous amyloidosis is a rare form of cutaneous amyloidosis and is characterized by an extracellular deposition of insoluble amyloid fibrils which are either primarily cutaneous or a manifestation of an underlying systemic amyloidosis. Biopsy of the lesion is mandatory for the diagnosis, and histopathology shows diffuse amyloid deposits with plasmacytic infiltration. Apple-green birefringence characteristic of amyloidosis is observed when stained with Congo red and viewed under polarized light. Read More

Lasers Med Sci 2022 Mar 6;37(2):799-813. Epub 2021 Oct 6.

Faculty of Medicine, Kerman University of Medical Sciences, Haft Bagh Alavi Blvd, Postal Code: 7616913555, Kerman, Iran.

With the investigation of the efficacy of laser therapy in primary localized amyloidosis(PLCA) only recently starting to materialize, we aimed to review the currently available studies of laser therapy in the management of the disease. We searched PubMed, Scopus, Embase, Web of Science, Cochrane, and ProQuest online databases with a specified search strategy, assessed the quality of each study, and then extracted the eligible data. Five RCTs(randomized controlled trials), one non-randomized controlled trial, three case series, and nine case reports(18 in total) were included. Read More

Dermatology 2022 15;238(3):579-586. Epub 2021 Sep 15.

Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.

Background: Primary localized cutaneous amyloidosis (PLCA) is defined by the deposition of amyloid protein in the skin without systemic involvement. There are four subtypes of PLCA: lichen amyloidosis (LA), macular amyloidosis (MA), biphasic amyloidosis (BA), and nodular amyloidosis (NA). PLCA occurs most frequently in Latin Americans and Asians. Read More

Open Life Sci 2021 9;16(1):781-784. Epub 2021 Aug 9.

Department of Scientific Research, Shanghai Xuhui Center Hospital, Shanghai 200031, China.

Primary skin amyloidosis is a chronic skin disease in which amyloid deposits in the normal skin tissues without involving other organs. At present, the causes and mechanisms of morbidity have not been fully elucidated. There are few clinical reports of nodular skin amyloidosis, and the domestic reports are mostly limited cases. Read More

Dermatopathology (Basel) 2021 Jul 2;8(3):229-235. Epub 2021 Jul 2.

Dermatology Clinic, Department Medical Sciences and Public Health, University of Cagliari, 09124 Cagliari, Italy.

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. Read More

Dermatol Pract Concept 2021 May 20;11(3):e2021057. Epub 2021 May 20.

Department of Dermatology, S N Medical College, Bagalkot, Karnataka, India.

Background: Primary localized cutaneous amyloidosis (PLCA) causes extracellular proteinaceous deposits in skin. It is clinically divided into macular amyloidosis, lichen amyloidosis and nodular amyloidosis. Atypical presentations of PLCA make the diagnosis challenging, requiring biopsy to confirm amyloid deposition in the upper papillary dermis. Read More

Intern Med 2021 Aug 15;60(15):2513-2514. Epub 2021 Feb 15.

Department of Dermatology, National Center for Global Health and Medicine, Japan.

Cureus 2019 Oct 8;11(10):e5864. Epub 2019 Oct 8.

Dermatology, St. Joseph Dermatopathology, Houston, USA.

Primary localized cutaneous amyloidosis (PLCA) occurs when amyloid is deposited only within the skin and there is no evidence of systemic involvement. Nodular amyloidosis is the rarest subtype of PLCA. It typically involves the acral regions but can sometimes present on the head and neck. Read More

Scand J Rheumatol 2020 Mar 3;49(2):159-160. Epub 2019 Oct 3.

Department of Rheumatology, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK.

Amyloid 2019 Dec 18;26(4):225-233. Epub 2019 Sep 18.

Department of Diagnostic Pathology, Yamaguchi University Hospital , Ube , Japan.

Localized nodular deposits of AL amyloid are seen in different tissues/organs; however, the pathogenesis of this form of amyloidosis remains unclear. Recently, Sjögren syndrome combined with localized nodular AL amyloidosis has been noted. Here, we report Sjögren syndrome cases showing multifocal nodular AL amyloidosis and the followed benign course. Read More

Dermatol Pract Concept 2019 Jul 31;9(3):235-236. Epub 2019 Jul 31.

Serviço de Dermatologia, Universidade de Mogi das Cruzes, São Paulo, Brazil.

Am J Dermatopathol 2019 Dec;41(12):945-947

Hospital Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS, Brazil.

Primary localized cutaneous nodular amyloidosis is a rare plasma cell dyscrasia in which an amorphous material consisting of light chain amyloid is produced and deposited in the dermis, with varied clinical presentation. We describe the case with unusual and tumor lush clinical presentation in the face with no progression to systemic disease and no evidence of extracutaneous commitment. Read More

Skinmed 2019 29;17(2):131-133. Epub 2019 May 29.

Baylor College of Medicine, Houston, TX.

A 41-year-old man presented with an asymptomatic, slowly enlarging plaque on the lower aspect of his left cheek, present for approximately 4 months. His past medical history was remarkable for allergic rhinitis. Three years before, he had had a nearly identical lesion surgically excised by Mohs method at the same site. Read More

Cureus 2019 Jan 14;11(1):e3879. Epub 2019 Jan 14.

Dermatology, San Diego Family Dermatology, San Diego, USA.

Amyloid deposition has been observed in tissue specimens of basal cell carcinomas. A 68-year-old man with a nodular basal cell carcinoma on his left arm near the elbow is described; microscopic evaluation of the biopsy tissue specimen shows not only nodular aggregates and strands of atypical basaloid tumor cells but also marked deposition of amorphous amyloid material in the stroma between the aggregates of basal cell carcinoma. Including the man in this report, there are additional individual descriptions of patients whose basal cell carcinomas have amyloid deposits in the adjacent stroma or within the tumor aggregates or both. Read More

J Am Acad Dermatol 2018 Oct 19;79(4):736-745. Epub 2018 May 19.

Department of Dermatology, University of Connecticut, Farmington, Connecticut. Electronic address:

Sjögren syndrome (SS) is an autoimmune connective tissue disorder (CTD) that principally affects the lacrimal and salivary glands. Although SS is 1 of the 3 most common autoimmune CTDs alongside systemic lupus erythematosus and progressive systemic sclerosis, it is the least researched CTD overall. SS poses a particular diagnostic challenge because it shares multiple clinical and immunologic features with other CTDs. Read More

Dermatol Online J 2017 Aug 15;23(8). Epub 2017 Aug 15.

Department of Dermatology and STD, Lady Hardinge Medical College. New Delhi, India.

Primary localized cutaneous amyloidosis refers to a group of disorders characterized by deposition of amyloid in the dermis without any systemic involvement. It comprises the following clinical types: macular, lichenoid, nodular, and biphasic. There are also rare variants such as amyloidosis cutis dyscromica and poikiloderma-like cutaneous amyloidosis. Read More

Acta Derm Venereol 2018 01;98(1):144-145

Dermatology Department, Hospital de la Santa Creu i Sant Pau, Carrer de Sant Quintí, 89, ES-08026 Barcelona, Spain.

Clin Rev Allergy Immunol 2017 Dec;53(3):357-370

Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Via A. Manzoni 56, 20089 Rozzano, Milan, Italy.

Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases. Further, xerophtalmia (also known as dry eye) frequently associated with keratoconjunctivitis sicca cumulatively affects approximately 10-30% of the general population with increasing incidence with age and is more frequently secondary to non-autoimmune diseases. Read More

J Cutan Pathol 2017 Sep 19;44(9):801-804. Epub 2017 Jul 19.

Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine, Miami, Florida.

Soft tissue amyloidoma is a rare condition that presents primarily in the abdomen and/or mediastinum and more uncommonly on the extremities. Soft tissue amyloidomas on the extremities have been associated with chronic inflammation, particularly when accompanied by AA-type amyloid deposition as seen in local trauma, surgery, hypertension and diabetes. To our knowledge, this is the first reported case of nodular cutaneous amyloidoma in the setting of systemic and cutaneous sarcoidosis. Read More

Ann Dermatol 2017 Jun 11;29(3):349-351. Epub 2017 May 11.

Department of Dermatology, The Catholic University of Korea, Uijeongbu St. Mary's Hospital, Uijeongbu, Korea.

Int J Womens Dermatol 2017 Jun 11;3(2):91-95. Epub 2017 Jan 11.

Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, IA.

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. Read More

Am J Clin Dermatol 2017 Oct;18(5):629-642

Department of Dermatology, University Hospital Jena, Erfurter Straße 35, Jena, 07743, Germany.

Background: Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. Lichen amyloidosis, macular amyloidosis, and (primary localized cutaneous) nodular amyloidosis are different subtypes of PLCA.

Objective: The aim of this study was to review the current reported treatment options for PLCA. Read More

JAAD Case Rep 2016 Sep 29;2(5):373-376. Epub 2016 Sep 29.

Mid North Coast Area Health Service, Sydney.

Case Rep Dermatol 2016 May-Aug;8(2):193-6. Epub 2016 Jul 12.

Department of Dermatology, University Medical Center Regensburg, Regensburg, Germany.

A 52-year-old woman presented with a large partially yellow and erythematous tumor on her right temple. She reported that it had grown over the last 4 years. Regional lymph nodes were impalpable. Read More

Case Rep Dermatol 2016 Jan-Apr;8(1):22-5. Epub 2016 Feb 3.

Department of Dermatology, University Medical Center Regensburg, Germany.

A 79-year-old man presented with a large tumor on the left side of his head, which had grown over 5 years. Regional lymph nodes were impalpable and computed tomography revealed no signs of bone infiltration. Histology showed that the entire dermis was filled with amorphous eosinophilic material. Read More

Ann Dermatol Venereol 2016 Feb 24;143(2):134-8. Epub 2015 Dec 24.

Service de dermatologie, hôpital Saint-André, CHU de Bordeaux, rue Jean-Burguet, 33076 Bordeaux, France. Electronic address:

Background: 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. Read More

Am J Dermatopathol 2015 Nov;37(11):e129-33

Departments of *Pathology, and †Orthopedics, University of Massachusetts Medical School, Worcester, MA.

Primary, localized cutaneous amyloidosis includes macular, lichen, and nodular (tumefactive) types in which the amyloid deposits are limited to the dermis without systemic involvement. The material in lichen and macular amyloidosis is derived from epidermal keratinocytes [keratinocyte-derived amyloid (AK)], whereas that in nodular amyloidosis is derived from immunoglobulin light-chains amyloid (AL). Primary, localized cutaneous nodular amyloidosis (PLCNA) is a form of primary, localized cutaneous amyloidosis that has been associated with a risk of progression to systemic amyloidosis. Read More

Cutis 2015 Jun;95(6):E24-7

Department of Dermatology, Southwest Hospital, Third Military Medical University, Chongqing, 400038 China.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare form of cutaneous amyloidosis. We report the case of a 65-year-old woman with multiple asymptomatic discrete nodules and atrophic plaques on the thighs of 4 years' duration that had increased in number and size. Results of extensive clinical, histologic, and laboratory evaluation showed no evidence of systemic amyloidosis or myeloma. Read More