Amyloidosis, Nodular Localized Cutaneous Clinical Presentation

  • Author: Lauren Biesbroeck; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 5, 2010
 

History

  • 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.
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Physical

  • Firm nodules can present anywhere on the skin, including the face, scalp, extremities, trunk, and genitalia.[10, 11, 12]
  • Nodules vary from a few millimeters to a few centimeters.
  • Nodules appear pink to brown or red.
  • Overlying 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.
  • 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.
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Causes

The cause of nodular localized cutaneous amyloidosis is not known, although the amyloid protein is derived from a localized infiltrate of plasma cells.

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Contributor Information and Disclosures
Author

Lauren Biesbroeck  Washington University in St Louis School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Gregory J Raugi, MD, PhD  Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; 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.

Specialty Editor Board

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, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey

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

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

References

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  2. 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. [Medline].

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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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