Anetoderma 

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

Background

Anetoderma (anetos, Greek for slack) is a benign condition with focal loss of dermal elastic tissue, resulting in localized areas of flaccid or herniated saclike skin. The condition has been reported under various names, including macular atrophy and dermatitis atrophicans maculosa.

Historically, idiopathic lesions were classified based on a clinically inflammatory (Jadassohn-Pellizzari) or noninflammatory (Schweninger-Buzzi) onset. However, both types of lesions may be found in the same patient, and they are histologically similar. Currently, anetoderma is classified as either primary anetoderma, which is an idiopathic occurrence of atrophic lesions in areas of skin that appear normal prior to the onset of atrophy, or secondary anetoderma, which is preceded by an inflammatory or other skin pathology in the same location. Both types may be associated with systemic diseases.

Next

Pathophysiology

The exact cause of anetoderma is unknown. Possible explanations for loss of elastic tissue include defective elastin synthesis, uncontrolled production of elastolytic enzymes, loss of elastolytic enzyme inhibitors, elastophagocytosis, or degeneration of elastic fibers secondary to local ischemia induced by microthromboses in dermal vessels.[1] Some investigators have proposed a possible common epitope between elastic fibers and phospholipids as an explanation for an autoimmune-mediated process.[1]

Elastolytic activity has been shown in matrix metalloproteinases (MMPs),[2] cathepsins, and elastases. Inflammatory cells (macrophages and neutrophils) are the most important producers of these enzymes. Many different proinflammatory stimuli can cause them to produce elastolytic enzymes. These stimuli include, but are not limited to, the deposition of autoimmune complexes, infections, hypoxia/re-oxygenation, and physiochemical injuries.

Elastolytic enzymes may be directly responsible for elastin degradation. Alternatively, they may act indirectly by modulating other inflammatory events, such as proteolytic activation of latent cytokines, which, in turn, may regulate the activity of other undiscovered elastolytic enzymes. Known elastolytic enzyme inhibitors include serine proteinase inhibitors (serpins) and tissue inhibitors of metalloproteinase (TIMPs). An imbalance of the elastolytic enzymes and their inhibitors may change the rate of elastin turnover in affected areas, resulting in the visible lesions.

Previous
Next

Epidemiology

Frequency

United States

The exact incidence of anetoderma is not known, but secondary anetoderma is probably more common than the primary form. Familial anetoderma is uncommon, with only 12 families reported in the literature.[3, 4, 5, 6] Inheritance may be autosomal dominant, autosomal recessive, or undefined.

International

Secondary anetoderma may be more frequent in Central Europe than in North America, paralleling the incidence of acrodermatitis chronica atrophicans. This suggests that Borrelia species may be involved.

Mortality/Morbidity

Primary anetoderma is usually asymptomatic and not associated with mortality.

Sex

Primary anetoderma occurs slightly more frequently in women than in men.

Age

Most patients with primary anetoderma present between the second and fourth decade of life, although much younger[7, 8, 9] and older ages of onset have been reported.

Previous
Proceed to Clinical Presentation
 
 
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.

References

  1. Weinstein S, Piette W. Cutaneous manifestations of antiphospholipid antibody syndrome. Hematol Oncol Clin North Am. Feb 2008;22(1):67-77, vi. [Medline].

  2. Ghomrasseni S, Dridi M, Gogly B, Bonnefoix M, Vabres P, Venencie PY, et al. Anetoderma: an altered balance between metalloproteinases and tissue inhibitors of metalloproteinases. Am J Dermatopathol. Apr 2002;24(2):118-29. [Medline].

  3. Patrizi A, Neri I, Virdi A, Misciali C, D'acunto C. Familial anetoderma: a report of two families. Eur J Dermatol. Jul 1 2011;[Medline].

  4. Friedman SJ, Venencie PY, Bradley RR, Winkelmann RK. Familial anetoderma. J Am Acad Dermatol. Feb 1987;16(2 Pt 1):341-5. [Medline].

  5. Thomas JE, Mehregan DR, Holland J, Mehregan DA. Familial anetoderma. Int J Dermatol. Jan 2003;42(1):75-7. [Medline].

  6. Zellman GL, Levy ML. Congenital anetoderma in twins. J Am Acad Dermatol. Mar 1997;36(3 Pt 1):483-5. [Medline].

  7. Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol. May 2010;146(5):565-7. [Medline].

  8. Karrer S, Szeimies RM, Stolz W, Landthaler M. Primary Anetoderma in children: report of two cases and literature review. Pediatr Dermatol. Sep-Oct 1996;13(5):382-5. [Medline].

  9. Taïeb A, Dufillot D, Pellegrin-Carloz B, Calabet A, Clementy J, Guillard JM, et al. Postgranulomatous anetoderma associated with Takayasu's arteritis in a child. Arch Dermatol. Jun 1987;123(6):796-800. [Medline].

  10. Zaki I, Scerri L, Nelson H. Primary anetoderma: phagocytosis of elastic fibres by macrophages. Clin Exp Dermatol. Sep 1994;19(5):388-90. [Medline].

  11. Hodak E, Shamai-Lubovitz O, David M, Hazaz B, Katzenelson-Weissman V, Lahav M, et al. Immunologic abnormalities associated with primary anetoderma. Arch Dermatol. Jun 1992;128(6):799-803. [Medline].

  12. Staiger H, Saposnik M, Spiner RE, Schroh RG, Corbella MC, Hassan ML. Primary anetoderma: a cutaneous marker of antiphospholipid antibodies. Skinmed. May-Jun 2011;9(3):168-71. [Medline].

  13. de Souza EM, Daldon PE, Cintra ML. Anetoderma associated with primary antiphospholipid syndrome. J Am Acad Dermatol. May 2007;56(5):881-2. [Medline].

  14. Hodak E, David M. Primary anetoderma and antiphospholipid antibodies--review of the literature. Clin Rev Allergy Immunol. Apr 2007;32(2):162-6. [Medline].

  15. Herrero-Gonzalez JE, Herrero-Mateu C. Primary anetoderma associated with primary Sjogren's syndrome. Lupus. 2002;11(2):124-6. [Medline].

  16. Brenner S, Nemet P, Legum C. Jadassohn-type anetoderma in association with keratoconus and cataract. Ophthalmologica. 1977;174(4):181-4. [Medline].

  17. Larsen DA, Sommerlund M, Bek T. Anetoderma. A possible new risk factor for subretinal neovascularization. Acta Ophthalmol. Feb 2011;89(1):e95. [Medline].

  18. Brenner S, Gazit E, Lieberman M, Ilie B, Krakowski A. Jadassohn-type anetoderma in association with protrusion of the teeth. Dermatologica. 1980;161(5):334-9. [Medline].

  19. Kasper RC, Wood GS, Nihal M, LeBoit PE. Anetoderma arising in cutaneous B-cell lymphoproliferative disease. Am J Dermatopathol. Apr 2001;23(2):124-32. [Medline].

  20. Jubert C, Cosnes A, Clerici T, Gaulard P, Andre P, Revuz J, et al. Sjogren's syndrome and cutaneous B cell lymphoma revealed by anetoderma. Arthritis Rheum. Jan 1993;36(1):133-4. [Medline].

  21. Zattra, E, B. Pigozzi, et al. Anetoderma in cutaneous marginal-zone B-cell lymphoma. Clin Exp Dermatol. 2009;34(8):945-948.

  22. Ferrara G, Cusano F, Robson A, Stefanato CM. Primary cutaneous marginal zone B-cell lymphoma with anetoderma: spontaneous involution plus de novo clonal expansion. J Cutan Pathol. Apr 2011;38(4):342-5. [Medline].

  23. Peterman A, Scheel M, Sams WM Jr, Pandya AG. Hereditary anetoderma. J Am Acad Dermatol. Dec 1996;35(6):999-1000. [Medline].

  24. de Souza EM, Ayres Vallarelli AF, Cintra ML, Vetter-Kauczok CS, Brocker EB. Anetodermic pilomatricoma. J Cutan Pathol. Aug 18 2008;[Medline].

  25. Clement M, du Vivier A. Anetoderma secondary to syphilis. J R Soc Med. Mar 1983;76(3):223-4. [Medline].

  26. Crone AM, James MP. Acquired linear anetoderma following angular cheilitis. Br J Dermatol. May 1998;138(5):923-4. [Medline].

  27. Cockayne SE, Gawkrodger DJ. Hamartomatous congenital melanocytic nevi showing secondary anetoderma-like changes. J Am Acad Dermatol. Nov 1998;39(5 Pt 2):843-5. [Medline].

  28. Jubert C, Cosnes A, Wechsler J, Andre P, Revuz J, Bagot M. Anetoderma may reveal cutaneous plasmacytoma and benign cutaneous lymphoid hyperplasia. Arch Dermatol. Mar 1995;131(3):365-6. [Medline].

  29. Sheehan DJ, Madkan V, Strickling WA, Peterson CM. Atrophic dermatofibrosarcoma protuberans: a case report and reappraisal of the literature. Cutis. Oct 2004;74(4):237-42. [Medline].

  30. Ozkan S, Fetil E, Izler F, Pabucçuoglu U, Yalcin N, Gunes AT. Anetoderma secondary to generalized granuloma annulare. J Am Acad Dermatol. Feb 2000;42(2 Pt 2):335-8. [Medline].

  31. Daoud MS, Dicken CH. Anetoderma after hepatitis B immunization in two siblings. J Am Acad Dermatol. May 1997;36(5 Pt 1):779-80. [Medline].

  32. Ruiz-Rodriguez R, Longaker M, Berger TG. Anetoderma and human immunodeficiency virus infection. Arch Dermatol. May 1992;128(5):661-2. [Medline].

  33. Trevisan G, Padovan C, Scaini MT, Cinco M, Floris R, Bonin S. Anetoderma associated with lyme disease: a case report. Acta Derm Venereol. 2008;88(5):536-8. [Medline].

  34. Shalders K, Ilchyshyn A, Walzman M. Secondary anetoderma following molluscum contagiosum infection. Acta Derm Venereol. 2003;83(6):461-2. [Medline].

  35. Requena L, Gonzalez-Guerra E, Angulo J, DeVore AE, Sangueza OP. Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides. Br J Dermatol. Jan 2008;158(1):157-62. [Medline].

  36. Chaby G, Viseux V, Chatelain D, Denoeux JP, Lok C. [Myxofibrosarcoma associated with anetoderma]. Ann Dermatol Venereol. Jan 2006;133(1):35-7. [Medline].

  37. Velez D, Reina Duran T, Perez-Gala S, Fernandez JF. Rosetoid schwannoma (neuroblastoma-like) in association with an anetoderma. J Cutan Pathol. Aug 2006;33(8):573-6. [Medline].

  38. Johnson WC. Wilson's disease and penicillamine-induced anetoderma. Arch Dermatol. 1977;113:976.

  39. Hirschel-Scholz S, Salomon D, Merot Y, Saurat JH. Anetodermic prurigo nodularis (with Pautrier's neuroma) responsive to arotinoid acid. J Am Acad Dermatol. Aug 1991;25(2 Pt 2):437-42. [Medline].

  40. Lee WJ, Yang JH, Chang SE, Lee MW, Choi JH, Moon KC. Secondary anetoderma overlying schwannoma. Acta Derm Venereol. 2009;89(2):219-20. [Medline].

  41. Prizant TL, Lucky AW, Frieden IJ, Burton PS, Suarez SM. Spontaneous atrophic patches in extremely premature infants. Anetoderma of prematurity. Arch Dermatol. Jun 1996;132(6):671-4. [Medline].

  42. Al Buainain H, Allam M. Anetoderma: Is It a Sign of Autoimmunity?. Case Rep Dermatol. 2009;1(1):100-104. [Medline].

  43. Bergman R, Friedman-Birnbaum R, Hazaz B, Cohen E, Munichor M, Lichtig C. An immunofluorescence study of primary anetoderma. Clin Exp Dermatol. Mar 1990;15(2):124-30. [Medline].

  44. Reiss F, Linn E. The therapeutic effect of epsilon-aminocaproic acid on anetoderma Jadassohn. Dermatologica. 1973;146(6):357-60. [Medline].

  45. Braun RP, Borradori L, Chavaz P, Masouye I, French L, Saurat JH. Treatment of primary anetoderma with colchicine. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):1002-3. [Medline].

  46. Aghaei S, Sodaifi M, Aslani FS, Mazharinia N. An unusual presentation of anetoderma: a case report. BMC Dermatol. Aug 19 2004;4:9. [Medline].

  47. Gibson GE, Su WP, Pittelkow MR. Antiphospholipid syndrome and the skin. J Am Acad Dermatol. Jun 1997;36(6 Pt 1):970-82. [Medline].

  48. Kalogeromitros D, Gregoriou S, Makris M, Georgala S, Kempuraj D, Theoharides TC. Secondary anetoderma associated with mastocytosis. Int Arch Allergy Immunol. 2007;142(1):86-8. [Medline].

  49. Kim JM, Su WP. Mid dermal elastolysis with wrinkling. Report of two cases and review of the literature. J Am Acad Dermatol. Feb 1992;26(2 Pt 1):169-73. [Medline].

  50. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired disorders of elastic tissue: Part II. decreased elastic tissue. J Am Acad Dermatol. Aug 2004;51(2):165-85; quiz 186-8. [Medline].

  51. Roberts NM, Farrell A, Woodrow D, Leibowitch M, Staughton RC. Anetoderma of Jadassohn-Pellizzari. J R Soc Med. Oct 1995;88(10):599P-600P. [Medline].

  52. Shames BS, Nassif A, Bailey CS, Saltzstein SL. Secondary anetoderma involving a pilomatricoma. Am J Dermatopathol. Oct 1994;16(5):557-60. [Medline].

  53. Stephansson EA, Niemi KM. Antiphospholipid antibodies and anetoderma: are they associated?. Dermatology. 1995;191(3):204-9. [Medline].

  54. Venencie PY, Winkelmann RK, Moore BA. Anetoderma. Clinical findings, associations, and long-term follow-up evaluations. Arch Dermatol. Aug 1984;120(8):1032-9. [Medline].

 
Previous
Next
 
Multiple lesions of anetoderma.
Close-up view of a single lesion of anetoderma.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.