History

The patient history may include the following:

Nodular localized cutaneous amyloidosis lesions usually are asymptomatic.
Patients can present with single or multiple lesions.
In some reports, lesions were present for several years before patients sought medical attention.
Troublesome aspects of nodular localized cutaneous amyloidosis primarily result from patient concerns about appearance, although plaques eventually fissured in one patient in whom the plantar aspects of the feet were affected.
Up to 25% of reported cases of NLCA have been in patients with Sjögren syndrome.^[15]
In some cases, patients have given a history of preceding trauma.^{[4, 16, 17]}

Physical Examination

Physical examination may reveal the following:

Firm nodules can present at any cutaneous site including the face, scalp, extremities, trunk, and genitalia; however, acral areas are preferentially involved.^{[18, 19, 20, 21]}
Nodules vary from a few millimeters to a few centimeters and may coalesce to form larger plaques.
Disseminated cases have been described.^[22]
Nodules appear pink to brown or red.
Underlying epidermal atrophy has been described.
Other terms that describe the various lesions of nodular localized cutaneous amyloidosis include waxy, purpuric, yellowish, or bullous.
Lesions tend not to ulcerate, although a case of localized ulcerative nodular amyloidosis with deep involvement was described.^[23]
Nodular localized cutaneous amyloidosis lacks extracutaneous findings by definition; however, one patient reported to have nodular localized cutaneous amyloidosis had amyloid deposits in the rectum.
Macroglossia, a typical feature of systemic amyloidosis, is not seen in nodular localized cutaneous amyloidosis.

Differential Diagnoses

Dokic Y, Subrt P, Tschen J. A rare presentation of nodular amyloidosis on the lower back. Cureus. 2019 Oct 8. 11 (10):e5864. [QxMD MEDLINE Link].
Hagari Y, Mihara M, Hagari S. Nodular localized cutaneous amyloidosis: detection of monoclonality of infiltrating plasma cells by polymerase chain reaction. Br J Dermatol. 1996 Oct. 135(4):630-3. [QxMD MEDLINE Link].
Grunewald K, Sepp N, Weyrer K. Gene rearrangement studies in the diagnosis of primary systemic and nodular primary localized cutaneous amyloidosis. J Invest Dermatol. Oct 1991. 97(4):693-6. [QxMD MEDLINE Link].
Kalajian AH, Waldman M, Knable AL. Nodular primary localized cutaneous amyloidosis after trauma: a case report and discussion of the rate of progression to systemic amyloidosis. J Am Acad Dermatol. Aug 2007. 57(2 Suppl):S26-9. [QxMD MEDLINE Link].
Woollons A, Black MM. Nodular localized primary cutaneous amyloidosis: a long-term follow-up study. Br J Dermatol. Jul 2001. 145(1):105-9. [QxMD MEDLINE Link].
Moon AO, Calamia KT, Walsh JS. Nodular amyloidosis: review and long-term follow-up of 16 cases. Arch Dermatol. Sep 2003. 139(9):1157-9. [QxMD MEDLINE Link].
Inazumi T, Hakuno M, Yamada H. Characterization of the amyloid fibril from primary localized cutaneous nodular amyloidosis associated with Sjögren's syndrome. Dermatology. 1994. 189(2):125-8. [QxMD MEDLINE Link].
Srivastava M. Primary cutaneous nodular amyloidosis in a patient with Sjögren's syndrome. J Drugs Dermatol. Mar 2006. 5(3):279-80. [QxMD MEDLINE Link].
Yoneyama K, Tochigi N, Oikawa A, Shinkai H, Utani A. Primary localized cutaneous nodular amyloidosis in a patient with Sjögren's syndrome: a review of the literature. J Dermatol. Feb 2005. 32(2):120-3. [QxMD MEDLINE Link].
Konishi A, Fukuoka M, Nishimura Y. Primary localized cutaneous amyloidosis with unusual clinical features in a patient with Sjögren's syndrome. J Dermatol. Jun 2007. 34(6):394-6. [QxMD MEDLINE Link].
Meijer JM, Schonland SO, Palladini G, et al. Sjögren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity?. Arthritis Rheum. Jul 2008. 58(7):1992-9. [QxMD MEDLINE Link].
Summers EM, Kendrick CG. Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature. Cutis. Jul 2008. 82(1):55-9. [QxMD MEDLINE Link].
Ueno M, Yamamoto T, Yamanaka M, Matsunaga T. Nodular amyloidosis in a patient with liver cirrhosis. Dermatol Online J. Jul 2011. 17(7):10. [QxMD MEDLINE Link].
Fujisawa T, Shu E, Ikeda T, Seishima M. Primary localized cutaneous nodular amyloidosis that appeared in a patient with severe atopic dermatitis. J Dermatol. Mar 2012. 39(3):312-3. [QxMD MEDLINE Link].
Wey SJ, Chen YM, Lai PJ, Chen DY. Primary sjögren syndrome manifested as localized cutaneous nodular amyloidosis. J Clin Rheumatol. Oct 2011. 17(7):368-70. [QxMD MEDLINE Link].
Lee DY, Kim YJ, Lee JY, Kim MK, Yoon TY. Primary localized cutaneous nodular amyloidosis following local trauma. Ann Dermatol. Nov 2011. 23(4):515-8. [QxMD MEDLINE Link].
Caruana D, McCusker S, Harper C, Bilsland D. Curious facial plaque diagnosed as nodular primary localised cutaneous amyloidosis. BMJ Case Rep. 2019 May 6. 12 (5):[QxMD MEDLINE Link].
Hicks BC, Weber PJ, Hashimoto K, Ito K, Koreman DM. Primary cutaneous amyloidosis of the auricular concha. J Am Acad Dermatol. Jan 1988. 18(1 Pt 1):19-25. [QxMD MEDLINE Link].
Mun KS, Pailoor J, Reddy SC. Primary localised deep cutaneous amyloidosis of the eyelid. Malays J Pathol. Dec 2005. 27(2):113-5. [QxMD MEDLINE Link].
Northcutt AD, Vanover MJ. Nodular cutaneous amyloidosis involving the vulva. Case report and literature review. Arch Dermatol. Apr 1985. 121(4):518-21. [QxMD MEDLINE Link].
Borowicz J, Shama L, Miller R. Nodular cutaneous amyloidosis. Skinmed. Sep-Oct 2011. 9(5):316-8. [QxMD MEDLINE Link].
Criado PR, Silva CS, Vasconcellos C, et al. Extensive nodular cutaneous amyloidosis: an unusual presentation. Br J Dermatol. Jul 2001. 145(1):105-9. [QxMD MEDLINE Link].
Fernandes C, Éthier V, Marouan S, Chababi-Atallah M, Veilleux M. Localized ulcerative nodular amyloidosis presenting as ulcerative panniculitis, an unusual clinical manifestation: A case report. SAGE Open Med Case Rep. 2019. 7:2050313X19890755. [QxMD MEDLINE Link].
Lien MH, Railan D, Nelson BR. The efficacy of dermabrasion in the treatment of nodular amyloidosis. J Am Acad Dermatol. Feb 1997. 36(2 Pt 2):315-6. [QxMD MEDLINE Link].
Hamzavi I, Lui H. Excess tissue friability during CO2 laser vaporization of nodular amyloidosis. Dermatol Surg. Sep 1999. 25(9):726-8. [QxMD MEDLINE Link].
Truhan AP, Garden JM, Roenigk HH Jr. Nodular primary localized cutaneous amyloidosis: immunohistochemical evaluation and treatment with the carbon dioxide laser. J Am Acad Dermatol. Jun 1986. 14(6):1058-62. [QxMD MEDLINE Link].
Lesiak A, Rakowski A, Brzezinska A, et al. Effective treatment of nodular amyloidosis with carbon dioxide laser. Cutan Med Surg. Sep-Oct 2012. 16(5):372-4. [QxMD MEDLINE Link].
Alster TS, Manaloto RM. Nodular amyloidosis treated with a pulsed dye laser. Dermatol Surg. Feb 1999. 25(2):133-5. [QxMD MEDLINE Link].
Tong PL, Walker WA, Glancy RJ, et al. Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide. Australas J Dermatol. Feb 2013. 54(1):e12-5. [QxMD MEDLINE Link].
Raymond J, Choi J. Nodular cutaneous amyloidosis effectively treated with intralesional methotrexate. JAAD Case Rep. 2016 Sep 29. 2(5):373-376. [QxMD MEDLINE Link]. [Full Text].
Bozikov K, Janezic T. Excision and split thickness skin grafting in the treatment of nodular primary localized cutaneous amyloidosis. Eur J Dermatol. May-Jun 2006. 16(3):315-6. [QxMD MEDLINE Link].
Breathnach SM. Amyloid and amyloidosis. J Am Acad Dermatol. Jan 1988. 18(1 Pt 1):1-16. [QxMD MEDLINE Link].
Carroll CB, Collison DW, Rodman OG Jr. Atrophic outpouchings of abdominal skin. Nodular cutaneous amyloidosis. Arch Dermatol. Feb 1996. 132(2):223-4, 226-7. [QxMD MEDLINE Link].
Helm TN, Danziger J, Helm KF. Bilateral plantar amyloidosis: a unique presentation of localized cutaneous amyloidosis. Cutis. Mar 1997. 59(3):142-4. [QxMD MEDLINE Link].
Horiguchi Y, Takahashi C, Imamura S. A case of nodular cutaneous amyloidosis. Amyloid production by infiltrating plasma cells. Am J Dermatopathol. Feb 1993. 15(1):59-63. [QxMD MEDLINE Link].
Huilgol SC, Ramnarain N, Carrington P, et al. Cytokeratins in primary cutaneous amyloidosis. Australas J Dermatol. May 1998. 39(2):81-5. [QxMD MEDLINE Link].
Kakani RS, Goldstein AE, Meisher I, Hoffman C. Nodular amyloidosis: case report and literature review. J Cutan Med Surg. Mar-Apr 2001. 5(2):101-4. [QxMD MEDLINE Link].
Masuda C, Hayashi M, Kameda Y, et al. Protein AL origin in amyloidosis cutis nodularis atrophicans. J Dermatol. Aug 1986. 13(4):280-4. [QxMD MEDLINE Link].
Masuda C, Mohri S, Nakajima H. Histopathological and immunohistochemical study of amyloidosis cutis nodularis atrophicans--comparison with systemic amyloidosis. Br J Dermatol. Jul 1988. 119(1):33-43. [QxMD MEDLINE Link].
Touart DM, Sau P. Cutaneous deposition diseases. Part I. Aug 1998. 39(2 Pt 1):149-71; quiz 172-4. [QxMD MEDLINE Link].
Trau H, Shpiro D, Schewach-Millet M, et al. Nodular cutaneous amyloidosis. Am J Dermatopathol. Aug 1991. 13(4):414-7. [QxMD MEDLINE Link].
Vestey JP, Tidman MJ, Mclaren KM. Primary nodular cutaneous amyloidosis--long-term follow-up and treatment. Clin Exp Dermatol. Mar 1994. 19(2):159-62. [QxMD MEDLINE Link].

Media Gallery

The bright pink homogeneous-appearing material seen is amyloid stained with Congo red. A distinguishing feature of amyloid in the skin is an affinity to take up Congo red stain.
Amyloid shows apple green when examined with polarized light.
This transmission electron micrograph of amyloid deposited in the tissue shows loosely interwoven straight filaments.

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Author

Nicholas V Nguyen, MD Director of Pediatric Dermatology, Akron Children's Hospital

Nicholas V Nguyen, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Tracy Funk, MD Fellow in Pediatric Dermatology, Department of Dermatology, The Children’s Hospital Colorado

Tracy Funk, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Academy of Pediatrics, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Catharine Lisa Kauffman, MD, FACP Georgetown Dermatology and Georgetown Dermpath

Catharine Lisa Kauffman, MD, FACP is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Royal Society of Medicine, Society for Investigative Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Acknowledgements

Lauren Biesbroeck Washington University in St Louis School of Medicine

Disclosure: Nothing to disclose. Marion C Miethke, MD Clinical Assistant Professor, Department of Internal Medicine, Section of Dermatology, University of Washington

Disclosure: Nothing to disclose.

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.