Anetoderma Clinical Presentation

  • Author: Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Sep 12, 2011
 

History

The initial primary lesions may be noticed as crops of 1–2 cm. asymptomatic erythematous macules, plaques, nodules, or wheals 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

Patients have multiple, one to several centimeter, circumscribed, skin-colored, grey-white or blue, wrinkled macules or patches that may bulge slightly like pouches and herniate into the skin upon palpation (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.

Anetoderma is one of several elastolytic disorders that include cutis laxa, mid dermal elastolysis, and granulomatous slack skin. Anetoderma is clinically recognized by well-demarcated sac-like bulging when pan-dermal elastolysis is present. If the elastolysis is only partial, bulging may not be appreciated. Acquired cutis laxa differs from anetoderma by its generally widespread sagging or lax skin. Mid-dermal elastolysis is characterized by selective loss of elastic fibers in the mid dermis. This results in widespread fine wrinkling with or without perifollicular papules. Granulomatous slack skin is a rare variant of cutaneous T-cell lymphoma manifesting as erythematous pendulous folds of lax skin. It is histologically characterized by granulomas with phagocytosis of elastic fibers.[10]

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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 of lupus erythematosus, and other autoimmune disorders, and primary anetoderma is often cited in the literature. This is due to the frequent finding of antinuclear antibodies, antiphospholipid antibodies, hypocomplementemia, thyroid autoantibodies, and/or a positive lupus band test. However, the American Rheumatism Association criteria for diagnosis of systemic lupus erythematosus are typically not met.

Studies have suggested a strong association between primary anetoderma and antiphospholipid antibodies, with or without antiphospholipid antibody syndrome.[11] 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.[12, 13, 14]

Reported associations with primary anetoderma include the following:

Secondary anetoderma has been associated with many conditions. Examples that have been cited multiple times in the literature include the following (listed alphabetically):

  • Acne
  • Acrodermatitis chronica atrophicans
  • Amyloidosis
  • Antiphospholipid antibody syndrome
  • B-cell lymphoma[19] (1 case with concomitant Sjögren syndrome[20] )
  • Benign cutaneous lymphoid hyperplasia
  • Cutaneous marginal zone B-cell lymphoma[21, 22]
  • Dermatofibroma
  • Hansen disease
  • Hereditary[23]
  • Insect bites
  • Lupus erythematosus
  • Lupus profundus
  • Lymphocytoma
  • Perifolliculitis
  • Pilomatrixoma[24]
  • Sarcoidosis
  • Syphilis[25]
  • Tuberculosis
  • Urticaria pigmentosa
  • Varicella

Secondary anetoderma associations that have been reported rarely in the literature include the following (listed alphabetically):

  • Angular cheilitis[26]
  • Congenital melanocytic nevi with hamartomatous features[27]
  • Cutaneous plasmacytoma[28]
  • Dermatofibrosarcoma protuberans[29]
  • Discoid lupus
  • Granuloma annulare[30]
  • Hepatitis B vaccination[31]
  • HIV disease[32]
  • Juvenile xanthogranuloma
  • Langerhans cell histiocytosis
  • Lyme disease[33]
  • Molluscum contagiosum[34]
  • Mycosis fungoides[35]
  • Myxofibrosarcoma[36]
  • Neuroblastomalike schwannoma[37]
  • Penicillamine use[38]
  • Pityriasis versicolor
  • Prurigo nodularis[39]
  • Recurrent deep vein thrombosis
  • Schwannoma[40]
  • Sjögren syndrome[20]
  • Takayasu arteritis[9]
  • Under electrodes in growth restricted preterm infants[7, 41]
  • Xanthoma

Some secondary anetoderma lesions only vaguely resemble idiopathic anetoderma clinically, despite showing a loss of dermal elastic fibers. Examples of such secondary lesions include those associated with angular cheilitis, HIV disease, pilomatrixoma, and prurigo nodularis.

Secondary anetodermal lesions are parainflammatory or appear at the same location following a primary skin process.

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Contributor Information and Disclosures
Author

Anne Elizabeth Laumann, MBChB, MRCP(UK), FAAD  Associate Professor, Department of Dermatology, 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, Illinois State Medical Society, Medical Dermatology Society, and Society for Investigative Dermatology

Disclosure: Abbott Grant/research funds Other; Regeneron Grant/research funds Other; Centocor Grant/research funds Other; OSI Grant/research funds Other; Celgene Grant/research funds Other; Lilly Grant/research funds Other

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, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Stephen C Ho, MD  Boulder Dermatology Clinic

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.

Specialty Editor Board

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.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and 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, 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, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Jenny Sun, MD, and Jin Mo Park, PhD, to the development and writing of this article.

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