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Anetoderma

Overview

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, anetoderma maculosa, and atrophia maculosa cutis. Primary lesions consist of localized areas of flaccid skin, which can be macular, papular, or depressed.

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 individual, 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, autoimmune, infectious, or neoplastic process. Both types may be associated with systemic diseases. A familial form that manifests as primary anetoderma has also been described.^[1]

Pathophysiology

The exact cause of anetoderma is unknown. Possible explanations for the 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.^[2]Some investigators have proposed a possible common epitope between elastic fibers and phospholipids as an explanation for an autoimmune-mediated process.^[2]

Elastolytic enzymes such as matrix metalloproteinases (MMPs) may be directly responsible for elastin degradation in anetoderma. 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 in levels of MMPs and TIMPs altering the rate of elastin turnover has been suggested as one explanation of the pathophysiology of anetoderma. Specifically, MMP-2 and MMP-9 have been suggested as possible culprits at the origin of elastic fiber destruction in anetoderma.^[3]

Furthermore, a 2016 study involving nine patients with idiopathic anetoderma found that the dermal expression of fibulin-4 (which binds tropoelastin and is involved in various aspects of elastic fiber development) was significantly decreased in anetoderma when compared with healthy controls. These findings suggest that in addition to elastolytic overactivity, altered reassembly of elastic fibers may also play a significant role in the pathogenesis anetoderma.^[4]

Frequency

The exact incidence of anetoderma is unknown, but secondary anetoderma is probably more common than the primary form. Familial anetoderma is uncommon, with only 12 families reported in the literature.^{[5, 6, 7, 8]}Inheritance may be autosomal dominant, autosomal recessive, or undefined.

Sex

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

Age

Primary anetoderma usually presents between the second and fourth decades of life, although patients of all ages have been reported to develop anetoderma.^{[9, 10, 11]}

Prognosis

Once developed, the lesions do not change over time.

Clinical Presentation

Patrizi A, Neri I, Virdi A, Misciali C, D'Acunto C. Familial anetoderma: a report of two families. Eur J Dermatol. 2011 Sep-Oct. 21(5):680-5. [Medline].
Weinstein S, Piette W. Cutaneous manifestations of antiphospholipid antibody syndrome. Hematol Oncol Clin North Am. 2008 Feb. 22(1):67-77, vi. [Medline].
Kim JE, Sohn KM, Woo YJ, Jeong KH, Kim M, Lee JD, et al. A Clinicoimmunohistopathologic Study of Anetoderma: Is Protruding Type More Advanced in Stage Than Indented Type?. J Immunol Res. 2016. 2016:4325463. [Medline].
Gambichler T, Reininghaus L, Skrygan M, Schulze HJ, Schaller J, Colato C, et al. Fibulin Protein Expression in Mid-dermal Elastolysis and Anetoderma: A Study of 23 Cases. Acta Derm Venereol. 2016 Jun 15. 96 (5):708-10. [Medline].
Patrizi A, Neri I, Virdi A, Misciali C, D'acunto C. Familial anetoderma: a report of two families. Eur J Dermatol. 2011 Jul 1. [Medline].
Friedman SJ, Venencie PY, Bradley RR, Winkelmann RK. Familial anetoderma. J Am Acad Dermatol. 1987 Feb. 16(2 Pt 1):341-5. [Medline].
Thomas JE, Mehregan DR, Holland J, Mehregan DA. Familial anetoderma. Int J Dermatol. 2003 Jan. 42(1):75-7. [Medline].
Zellman GL, Levy ML. Congenital anetoderma in twins. J Am Acad Dermatol. 1997 Mar. 36(3 Pt 1):483-5. [Medline].
Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol. 2010 May. 146(5):565-7. [Medline].
Karrer S, Szeimies RM, Stolz W, Landthaler M. Primary Anetoderma in children: report of two cases and literature review. Pediatr Dermatol. 1996 Sep-Oct. 13(5):382-5. [Medline].
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. 1987 Jun. 123(6):796-800. [Medline].
Maari C, Powell J. Atrophies of connective tissue. Jean L. Bolognia, Joseph L. Jorizzo, and Julie V. Schaffer. Dermatology. Third. Elsevier Health Sciences; 6/18/2012. 2: 1631-40.
Hodak E, Shamai-Lubovitz O, David M, Hazaz B, Katzenelson-Weissman V, Lahav M, et al. Immunologic abnormalities associated with primary anetoderma. Arch Dermatol. 1992 Jun. 128(6):799-803. [Medline].
Staiger H, Saposnik M, Spiner RE, Schroh RG, Corbella MC, Hassan ML. Primary anetoderma: a cutaneous marker of antiphospholipid antibodies. Skinmed. 2011 May-Jun. 9(3):168-71. [Medline].
de Souza EM, Daldon PE, Cintra ML. Anetoderma associated with primary antiphospholipid syndrome. J Am Acad Dermatol. 2007 May. 56(5):881-2. [Medline].
Hodak E, David M. Primary anetoderma and antiphospholipid antibodies--review of the literature. Clin Rev Allergy Immunol. 2007 Apr. 32(2):162-6. [Medline].
Goujon E, Beer F, Gay S, Sandre D, Gouyon JB, Vabres P. Anetoderma of prematurity: an iatrogenic consequence of neonatal intensive care. Arch Dermatol. 2010 May. 146(5):565-7. [Medline].
Herrero-Gonzalez JE, Herrero-Mateu C. Primary anetoderma associated with primary Sjogren's syndrome. Lupus. 2002. 11(2):124-6. [Medline].
Brenner S, Nemet P, Legum C. Jadassohn-type anetoderma in association with keratoconus and cataract. Ophthalmologica. 1977. 174(4):181-4. [Medline].
Larsen DA, Sommerlund M, Bek T. Anetoderma. A possible new risk factor for subretinal neovascularization. Acta Ophthalmol. 2011 Feb. 89(1):e95. [Medline].
O'Gorman SM, Sheahan K, Collins P. Cocaine, a culprit in anetoderma?. Clin Exp Dermatol. Aug 2015. 40:695-6. [Medline].
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].
Connor CJ, Shchelochkov OA, Ciliberto H. Anetoderma in a patient with terminal osseous dysplasia with pigmentary defects. Am J Med Genet A. Oct 2015. 2459-62. [Medline].
Kasper RC, Wood GS, Nihal M, LeBoit PE. Anetoderma arising in cutaneous B-cell lymphoproliferative disease. Am J Dermatopathol. 2001 Apr. 23(2):124-32. [Medline].
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. 1993 Jan. 36(1):133-4. [Medline].
Zattra, E, B. Pigozzi, et al. Anetoderma in cutaneous marginal-zone B-cell lymphoma. Clin Exp Dermatol. 2009. 34(8):945-948.
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. 2011 Apr. 38(4):342-5. [Medline].
Pezzetta S, Russo I, Brumana M, Marino F, Alaibac M. Exacerbation of a Primary Follicular Centre Cutaneous B-cell Lymphoma during Pregnancy and Resolution to Anetoderma After Delivery. Acta Derm Ve. 2016 Aug 23. 96(6):828-9. [Medline].
Lee SM, Kim YJ, Chang SE. Pinhole carbon dioxide laser treatment of secondary anetoderma associated with juvenile xanthogranuloma. Dermatol Surg. 2012 Oct. 38(10):1741-3. [Medline].
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].
de Souza EM, Ayres Vallarelli AF, Cintra ML, Vetter-Kauczok CS, Brocker EB. Anetodermic pilomatricoma. J Cutan Pathol. 2008 Aug 18. [Medline].
Clement M, du Vivier A. Anetoderma secondary to syphilis. J R Soc Med. 1983 Mar. 76(3):223-4. [Medline].
Emer J, Roberts D, Sidhu H, Phelps R, Goodheart H. Generalized Anetoderma after Intravenous Penicillin Therapy for Secondary Syphilis in an HIV Patient. J Clin Aesthet Dermatol. 2013 Aug. 6(8):23-8. [Medline].
Crone AM, James MP. Acquired linear anetoderma following angular cheilitis. Br J Dermatol. 1998 May. 138(5):923-4. [Medline].
Cockayne SE, Gawkrodger DJ. Hamartomatous congenital melanocytic nevi showing secondary anetoderma-like changes. J Am Acad Dermatol. 1998 Nov. 39(5 Pt 2):843-5. [Medline].
Sheehan DJ, Madkan V, Strickling WA, Peterson CM. Atrophic dermatofibrosarcoma protuberans: a case report and reappraisal of the literature. Cutis. 2004 Oct. 74(4):237-42. [Medline].
Ozkan S, Fetil E, Izler F, Pabucçuoglu U, Yalcin N, Gunes AT. Anetoderma secondary to generalized granuloma annulare. J Am Acad Dermatol. 2000 Feb. 42(2 Pt 2):335-8. [Medline].
Kang HS, Paek JO, Lee MW, Yu HJ, Kim JS. Anetoderma developing in generalized granuloma annulare in an infant. Ann Dermatol. 2014 Apr. 26(2):283-5. [Medline].
Ruiz-Rodriguez R, Longaker M, Berger TG. Anetoderma and human immunodeficiency virus infection. Arch Dermatol. 1992 May. 128(5):661-2. [Medline].
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].
Shalders K, Ilchyshyn A, Walzman M. Secondary anetoderma following molluscum contagiosum infection. Acta Derm Venereol. 2003. 83(6):461-2. [Medline].
Requena L, Gonzalez-Guerra E, Angulo J, DeVore AE, Sangueza OP. Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides. Br J Dermatol. 2008 Jan. 158(1):157-62. [Medline].
Chaby G, Viseux V, Chatelain D, Denoeux JP, Lok C. [Myxofibrosarcoma associated with anetoderma]. Ann Dermatol Venereol. 2006 Jan. 133(1):35-7. [Medline].
Velez D, Reina Duran T, Perez-Gala S, Fernandez JF. Rosetoid schwannoma (neuroblastoma-like) in association with an anetoderma. J Cutan Pathol. 2006 Aug. 33(8):573-6. [Medline].
Johnson WC. Wilson's disease and penicillamine-induced anetoderma. Arch Dermatol. 1977. 113:976.
Hirschel-Scholz S, Salomon D, Merot Y, Saurat JH. Anetodermic prurigo nodularis (with Pautrier's neuroma) responsive to arotinoid acid. J Am Acad Dermatol. 1991 Aug. 25(2 Pt 2):437-42. [Medline].
Velez MJ, Mesinkovska N, Bergfeld WF. Anetoderma arising in Reed syndrome: a case report and review of the literature. J Cutan Pathol. May 2015. [Medline].
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].
Cho S, Jung JY, Lee JH. Treatment of Anetoderma Occurring After Resolution of Stevens-Johnson Syndrome Using an Ablative 10,600-nm Carbon Dioxide Fractional Laser. Dermatol Surg. 2012 Jan 17. [Medline].
Ivanova II1, Kotzev IA, Atanassova MV, Gancheva DT, Pavlov SI, Krasnaliev IJ, et al. Wilson's disease in association with anetoderma. Clin J Gastroenterol. 2015 Feb. 8(1):52-6. [Medline].
Prizant TL, Lucky AW, Frieden IJ, Burton PS, Suarez SM. Spontaneous atrophic patches in extremely premature infants. Anetoderma of prematurity. Arch Dermatol. 1996 Jun. 132(6):671-4. [Medline].
Maffeis L, Pugni L, Pietrasanta C, Ronchi A, Fumagalli M, Gelmetti C, et al. Iatrogenic anetoderma of prematurity: a case report and review of the literature. Case Rep Dermatol Med. [Medline].
Daoud MS, Dicken CH. Anetoderma after hepatitis B immunization in two siblings. J Am Acad Dermatol. 1997 May. 36(5 Pt 1):779-80. [Medline].
Zaki I, Scerri L, Nelson H. Primary anetoderma: phagocytosis of elastic fibres by macrophages. Clin Exp Dermatol. 1994 Sep. 19(5):388-90. [Medline].
Al Buainain H, Allam M. Anetoderma: Is It a Sign of Autoimmunity?. Case Rep Dermatol. 2009. 1(1):100-104. [Medline].
Bergman R, Friedman-Birnbaum R, Hazaz B, Cohen E, Munichor M, Lichtig C. An immunofluorescence study of primary anetoderma. Clin Exp Dermatol. 1990 Mar. 15(2):124-30. [Medline].
Reiss F, Linn E. The therapeutic effect of epsilon-aminocaproic acid on anetoderma Jadassohn. Dermatologica. 1973. 146(6):357-60. [Medline].
Braun RP, Borradori L, Chavaz P, Masouye I, French L, Saurat JH. Treatment of primary anetoderma with colchicine. J Am Acad Dermatol. 1998 Jun. 38(6 Pt 1):1002-3. [Medline].
Wang K, Ross NA, Saedi N. Anetoderma treated with combined 595-nm pulsed-dye laser and 1550-nm non-ablative fractionated laser. J Cosmet Laser Ther. 2016. 18:38-40. [Medline].

Media Gallery

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

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Anetoderma

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Anetoderma

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