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Intern Med 2021 Feb 15. 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

Indian J Dermatol Venereol Leprol 2015 Jul-Aug;81(4):400-2

Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Indian J Dermatol 2015 Jan-Feb;60(1):105

Department of Pathology, J N Medical College, Belgaum, Karnataka, India.

A 55-year-old man presented with multiple, itchy papules and macules on the trunk and extremities. Histopathologic examination of biopsy specimens taken from three different lesions showed a subepidermal blister with amyloid deposits in the dermal papillae. No systemic disease or involvement of other organs was detected. Read More

J Eur Acad Dermatol Venereol 2016 Mar 16;30(3):469-70. Epub 2014 Nov 16.

Department of Dermatology, Affiliated Hospital of Guangdong Medical College, Zhanjiang, Guangdong, China.

J Pak Med Assoc 2014 May;64(5):579-82

Primary localized cutaneous amyloidosis (PLCA) is caused by the extracellular deposition of amyloid material in the skin without other cutaneous or systemic organ involvement. PCLA is classified into lichen, macular,and nodular amyloidosis. Macular amyloidosis and lichen amyloidosis are named as biphasic amyloidosis when they are concurrently seen in a patient. Read More

Indian J Dermatol 2014 May;59(3):307-8

Department of Dermatology, Kyoto Prefectural University of Medicine Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan. E-mail:

Clin Exp Dermatol 2014 Apr;39(3):344-7

Division of Dermatopathology, Department of Dermatology, University of Connecticut Health Center, Farmington, CT, USA.

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. Read More

Cutis 2014 Feb;93(2):89-94

59 MDSP/SG07D, 2200 Bergquist Dr, Ste 1, JBSA-Lackland, TX 78236, USA.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare disorder that manifests as the cutaneous formation of nodules composed of light-chain amyloid. Although the type of amyloid deposit is similar to primary systemic amyloidosis, there seems to be little, if any, crossover between the 2 diseases. Because reports of PLCNA are sparse, there is no established protocol for treating this disease. Read More

Dermatol Online J 2013 Dec 16;19(12):20711. Epub 2013 Dec 16.

New York University School of Medicine.

A 61-year-old man presented for evaluation of a bruise-like lesion of the right knee. He was found to have an ill-defined, light brown patch with focal areas of dark red and brown. The histopathologic diagnosis was consistent with amyloidosis. Read More