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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

Br J Dermatol 2014 Mar;170(3):730-4

Faculty and Division of Medicine, Department of Dermatology, Chelsea & Westminster Hospital, 369 Fulham Road, London, SW10 9NH, U.K.

Background: Primary cutaneous amyloidosis of the penis is a rare entity.

Objectives: We describe two cases of primary penile cutaneous amyloidosis presenting as localized lesions on the penis and not associated with systemic amyloidosis.

Methods: A comprehensive literature review of all published cases. Read More

Cutis 2013 Jun;91(6):271, 283-4

Roger Williams Medical Center, Providence, RI 02908, USA.

Int J Dermatol 2015 Jun 20;54(6):708-9. Epub 2013 Jun 20.

Department of Dermatology, Norfolk and Norwich University Hospital, Norwich, UK.

Australas J Dermatol 2013 Feb 31;54(1):e12-5. Epub 2011 May 31.

Sir Charles Gairdner Hospital, Hospital Avenue, Nedlands, WA 6009, Australia.

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. Read More

Am J Surg Pathol 2013 Mar;37(3):406-12

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.

Nodular pulmonary amyloidosis, a rare localized form of amyloidosis, has been associated with immunoglobulin light chains (AL type) and variably with low-grade lymphoma. The clinicopathologic features of 18 cases were investigated; 5 of 14 had autoimmune disease. In 14 cases monotypic plasma cells could be demonstrated by immunohistochemistry. Read More

J Cutan Med Surg 2012 Sep-Oct;16(5):372-4

Department of Dermatology, Medical University of Lodz, Lodz, Poland.

Background: Nodular amyloidosis is a rare form of localized cutaneous amyloidosis that is characterized by nodules located on the extremities, trunk, genitalia, or face. In treatment regimens, many approaches have been described, including carbon dioxide (CO2) laser therapy.

Objective: We present a case of a 60-year-old white male with a 20-year history of disseminated waxy, purpuric, yellowish, and bullous skin lesions on the trunk and extremities. Read More

Skinmed 2011 Sep-Oct;9(5):316-8

Largo Medical Center, Largo, FL, USA.

A 56-year-old white man presented with a lesion on the right shoulder. The lesion developed during a short period and recently became irritated with occasional bleeding and mild pruritus. The patient denied pain. Read More

Ann Dermatol 2011 Nov 3;23(4):515-8. Epub 2011 Nov 3.

Department of Dermatology, School of Medicine and Medical Research Institute, Chungbuk National University, Cheongju, Korea.

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. Read More

An Bras Dermatol 2011 Sep-Oct;86(5):987-90

Fundação Alfredo da Matta, Manaus, AM, Brasil.

Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Read More

J Clin Rheumatol 2011 Oct;17(7):368-70

Department of Dermatology, School of Medicine, Chung-Shan Medical University Hospital, Taichung, Taiwan.

Localized cutaneous nodular amyloidosis (LCNA) is the rarest type of cutaneous amyloidosis. Typically presenting as waxy nodules on the lower extremities, it demonstrates localized deposition of AL-type amyloid in immunohistologic study and is often associated with focal plasma cell proliferation. Sjögren syndrome, an autoimmune lymphoproliferative disorder, is characterized by keratoconjunctivitis sicca and xerostomia with lymphocytic infiltration of exocrine glands. Read More

Actas Dermosifiliogr 2012 Mar 31;103(2):161-2. Epub 2011 Aug 31.

Dermatol Online J 2011 Jul 15;17(7):10. Epub 2011 Jul 15.

Department of Dermatology, Tsuchiura Kyodo General Hospital, Ibaraki, Japan.

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. Read More

J Dermatol 2012 Mar 18;39(3):312-3. Epub 2011 Jul 18.

Int J Dermatol 2011 Apr;50(4):458-61

Department of Dermatology, Venereology and Allergology, Leipzig University Medical Center, Philipp Rosenthalstr, Leipzig, Germany.

Am J Med 2010 Oct;123(10):904-6

Department of Dermatology, Sydney Cancer Centre, University of Sydney at Royal Prince Alfred Hospital, Camperdown, NSW, Australia.

Int J Dermatol 2010 Feb;49(2):229-30

Am J Dermatopathol 2010 May;32(3):298-300

Department of Pathology, University of South Florida, College of Medicine, Tampa, FL 33612, USA.

Primary cutaneous amyloidosis, also known as nodular amyloidosis, is defined as deposition of amyloid light chain in the skin in the absence of a systemic cause of amyloidosis. Such amyloid is produced by a localized aggregate of clonal plasma cells. In contrast, secondary cutaneous amyloidosis is related to lesions such as squamous cell carcinoma, Bowen disease, basal cell carcinoma, and discoid lupus erythematosus, and has been shown in most cases to be derived from keratin epithelial elements. Read More

Actas Dermosifiliogr 2008 Oct;99(8):648-52

Servicio de Dermatología, Hospital Universitario La Paz, Madrid, España.

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. Read More

Cutis 2008 Jul;82(1):55-9

Department of Internal Medicine, Harvard Medical Faculty Physicians, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Read More

Arthritis Rheum 2008 Jul;58(7):1992-9

University of Groningen, Groningen, The Netherlands.

Objective: To report 8 patients with Sjögren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases.

Methods: The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated. Read More

Dtsch Tierarztl Wochenschr 2007 Dec;114(12):473-5

University of Georgia, College of Veterinary Medicine, Department of Pathology, Tifton 31793, USA.

Cutaneous amyloidosis occurs as primary localized form or secondary to systemic amyloidosis. In dogs, cutaneous amyloidosis is reported in association with localized plasma cell proliferation or cutaneous extramedullary plasmacytoma. Primary cutaneous amyloidosis is a very rare condition in dogs. Read More

J Am Acad Dermatol 2007 Aug;57(2 Suppl):S26-9

Division of Dermatology, University of Louisville, Kentucky, USA.

Nodular primary localized cutaneous amyloidosis (NPLCA) has been associated with progression to systemic amyloidosis. The reported estimate of 50% progression to systemic amyloidosis has come under scrutiny as recent studies have suggested a significantly lower rate. Still, it is essential to consider systemic amyloidosis after making the diagnosis of NPLCA and to follow up patients longitudinally for possible progression to systemic disease. Read More

Arch Dermatol 2007 Apr;143(4):535-40

Case Western Reserve University, Cleveland, Ohio, USA.

Eur J Dermatol 2006 May-Jun;16(3):315-6

J Eur Acad Dermatol Venereol 2005 Jul;19(4):481-3

Dermatology Unit, Hospital do Servidor Público Estadual de São Paulo, Brazil.

Amyloidosis is characterized by the deposition of a group of unrelated proteins leading to changes in tissue architecture and function. The nodular variant is the rarest form of the cutaneous amyloidoses. We report a patient with localized nodular amyloidosis without systemic amyloid involvement or paraproteinaemia after 6 years of follow-up. Read More

J Dermatol 2005 Feb;32(2):120-3

Department of Dermatology, School of Medicine, Chiba University, Japan.

We report a 53-year-old Japanese woman with multiple, red, and elastic soft nodules on the left waist, left thigh, and right lower leg. She had had polyclonal hyperglobulinemia for one year, rheumatoid arthritis for 13 years, and Sjögren's syndrome (SjS) for 18 years. Histochemical examination of the nodule on the left thigh revealed a deposition of amyloid by Congo red staining. Read More

J Eur Acad Dermatol Venereol 2004 Mar;18(2):224-6

J Am Acad Dermatol 2003 Aug;49(2):307-10

Deparment of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.

We report a unique case of a 69-year-old man who presented with a 1-year history of pink nodules and plaques limited to the anterior plantar surface of the right foot and a 30-year history of a callus on the right heel. Histologic examination findings of both areas showed deposits of amorphous, eosinophilic material and an infiltrate of plasma cells in the dermis. Congo red-stained deposits exhibited apple-green birefringence with polarized light. Read More