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Anetoderma Clinical Presentation

  • Author: Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 21, 2015
 

History

Initial lesions may be noticed as crops of 1-2 cm, asymptomatic erythematous macules, plaques, or nodules that enlarge over weeks, possibly reaching several centimeters in diameter. Lesions appear on the upper arms, the trunk, the thighs, and less commonly on the neck, face, and rarely on distal extremities. Comorbid lesions may already exist. End-stage lesions do not change over time, and new lesions may continue to develop over years.

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Physical

Primary lesions of anetoderma present as discrete, flaccid areas of slack skin, which may be depressed, macular, or papular. Lesions may have overlying wrinkled skin. Lesions may be isolated or multiple. Lesions may slightly bulge like pouches and herniate into the skin upon palpation, a sign referred to as the "buttonhole" sign by some authors.[11]  See the images below.

Multiple lesions of anetoderma. Multiple lesions of anetoderma.
Close-up view of a single lesion of anetoderma. Close-up view of a single lesion of anetoderma.

On pressure, a normal ring of surrounding skin is felt. The scalp, the palms, the soles, and the mucous membranes are usually spared. A few to hundreds of lesions may form. These may coalesce and become indistinguishable from acquired cutis laxa.

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Causes

Primary or idiopathic anetoderma originates from previously healthy skin with unknown pathogenesis. Various autoimmune, ocular, bony, cardiac, and other abnormalities have been reported with anetoderma, including the familial forms. Whether these findings are coincidental or related is unknown. Thus, a thorough history and a complete physical examination are essential.

The association with lupus erythematosus or other autoimmune disorders and primary anetoderma is well-established. This is owing to the frequent finding of antinuclear antibodies, antiphospholipid antibodies, hypocomplementemia, thyroid autoantibodies, and/or a positive lupus band test in patients who have anetoderma. However, criteria for diagnosis of systemic lupus erythematosus are typically not met.

Familial anetoderma is a rare entity, which has only been reported in 12 families to date. Anetoderma was limited to the skin in some cases, while others were associated with extracutaneous abnormalities, particularly bone and/or cranial nerve anomalies.[1]

Anetoderma of prematurity is yet another entity occurring in very-low-birth-weight infants in neonatal intensive care units. Lesions usually present on the trunk and have been linked to the use of monitoring leads, leading to local hypoxia secondary to pressure on immature skin, thereby representing an acquired disorder.[12]

Studies have suggested a strong association between primary anetoderma and antiphospholipid antibodies, with or without antiphospholipid antibody syndrome.[13] Primary anetoderma may appear years earlier than the classic signs of an autoimmune syndrome. Thus it is recommended to search for clinical and laboratory signs of an autoimmune disorder in every patient presenting with primary anetoderma.[14, 15, 16]

Reported associations with primary anetoderma include the following:

Secondary anetoderma has been associated with myriad inflammatory, autoimmune, infectious, and neoplastic conditions. Associations that have been well-documented in the literature include the following (listed alphabetically):

  • Acne
  • Acrodermatitis chronica atrophicans
  • Amyloidosis
  • Antiphospholipid antibody syndrome
  • B-cell lymphoma [21] (one case with concomitant Sjögren syndrome [22] )
  • Benign cutaneous lymphoid hyperplasia
  • Cutaneous marginal zone B-cell lymphoma [23, 24]
  • Dermatofibroma
  • Hansen disease
  • Herpes zoster [25]
  • Arthropod bites
  • Lupus: Systemic lupus erythematosus, discoid lupus, lupus profundus
  • Lyme disease [26]
  • Lymphocytoma cutis
  • Pilomatricoma [27]
  • Sarcoidosis
  • Syphilis [28, 29]
  • Tuberculosis
  • Urticaria pigmentosa
  • Varicella

Additional conditions that have been reported to be associated with secondary anetoderma in isolated case reports include the following (listed alphabetically):

  • Angular cheilitis [30]
  • Congenital melanocytic nevi with hamartomatous features [31]
  • Dermatofibrosarcoma protuberans [32]
  • Generalized granuloma annulare [33, 34]
  • HIV disease [35]
  • Juvenile xanthogranuloma
  • Langerhans cell histiocytosis
  • Mastocytosis [36]
  • Molluscum contagiosum [37]
  • Mycosis fungoides [38]
  • Myxofibrosarcoma [39]
  • Neuroblastomalike schwannoma [40]
  • Penicillamine [41]
  • Pityriasis versicolor
  • Prurigo nodularis [42]
  • Reed syndrome [43]
  • Schwannoma [44]
  • Sjögren syndrome [22]
  • Stevens-Johnson’s syndrome [45]
  • Takayasu arteritis [10]
  • Wilson disease [46]

Furthermore, external traumatic injuries that have been linked to the development of secondary anetoderma include the following:

  • Electrodes (monitoring electrodes in premature infants), also known as iatrogenic anetoderma of prematurity [8, 47, 48]
  • Hepatitis B vaccination [49]
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Contributor Information and Disclosures
Author

Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD Professor of Dermatology, Chief of General Dermatology, Director of the Collagen Vascular Disorders Clinic, Northwestern University, The Feinberg School of Medicine

Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology, British Association of Dermatologists, Chicago Dermatological Society, Chicago Medical Society, Illinois Dermatological Society, Illinois State Medical Society, Medical Dermatology Society, Society for Investigative Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Julia Sanger Minocha, MD Resident Physician, Department of Medicine, Northwestern University, The Feinberg School of Medicine

Julia Sanger Minocha, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, Phi Beta Kappa

Disclosure: Nothing to disclose.

Victoria Godinez-Puig, MD Resident Physician, Department of Dermatology, Northwestern University, The Feinberg School of Medicine

Victoria Godinez-Puig, MD is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, 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.

Acknowledgements

Peter Fritsch, MD Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria

Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Stephen C Ho, MD  is a member of the following medical societies: American Academy of Dermatology, American Contact Dermatitis Society, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, American Society of Cosmetic Dermatology and Aesthetic Surgery, and Colorado Medical Society

Disclosure: Nothing to disclose.

Jin Mo Park, PhD Assistant Professor, Cutaneous Biology Research Center, Massachusetts General Hospital, Harvard Medical School

Disclosure: Nothing to disclose.

Jenny Sun, MD Research Fellow, Cutaneous Biology Research Center, Massachusetts General Hospital, Harvard Medical School

Disclosure: Nothing to disclose.

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Multiple lesions of anetoderma.
Close-up view of a single lesion of anetoderma.
 
 
 
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