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Pseudo-Kaposi Sarcoma (Acroangiodermatitis) Clinical Presentation

  • Author: Zoltan Trizna, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jan 08, 2016
 

History

A history of venous stasis, arteriovenous shunt for hemodialysis, or a long-standing arteriovenous malformation is usually present. A limb prosthesis may be present. Patients occasionally experience pruritus and pain.

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Physical

Confluent, violaceous or brown-black papules cover large areas of the distal parts of the legs. Ulceration and bleeding are sometimes noted. Bilateral lesions are usually associated with chronic venous insufficiency, whereas unilateral lesions suggest an underlying vascular malformation. Note the images below.

The physical findings in this patient who is HIV nThe physical findings in this patient who is HIV negative remained the same over a 3-year period.
Lesions on the shin of a patient who is HIV negatiLesions on the shin of a patient who is HIV negative.
Classic Kaposi sarcoma on the foot of an elderly pClassic Kaposi sarcoma on the foot of an elderly patient who is HIV negative. Compare this photo to the clinical photos of acroangiodermatitis.
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Causes

Severe, chronic venous stasis and the insufficiency of the muscle pump most commonly result in an elevated capillary pressure. Other factors (eg, an arteriovenous shunt for hemodialysis) may increase venous stasis and lead to the formation of arteriovenous channels. A rare case of acroangiodermatitis associated with a congenital arteriovenous malformation of the leg was also described (Stewart-Bluefarb syndrome).

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Contributor Information and Disclosures
Author

Zoltan Trizna, MD, PhD Private Practice

Zoltan Trizna, MD, PhD is a member of the following medical societies: Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Ronald P Rapini, MD Professor and Chair, Department of Dermatology, The University of Texas MD Anderson Cancer Center; Distinguished Chernosky Professor and Chair of Dermatology, Professor of Pathology, University of Texas McGovern Medical School at Houston

Ronald P Rapini, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Society for Investigative Dermatology, Texas Medical Association

Disclosure: Received royalty from Elsevier publishers for independent contractor; May receive consulting fee from FDA panel for consulting in future, since I am on one of their committees, but at this time so far have received zero from FDA.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

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.

Additional Contributors

Shyam Verma, MBBS, DVD, FAAD Clinical Associate Professor, Department of Dermatology, University of Virginia School of Medicine; Adjunct Associate Professor, Department of Dermatology, State University of New York at Stonybrook School of Medicine; Adjunct Associate Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
  1. Gucluer H, Gurbuz O, Kotiloglu E. Kaposi-like acroangiodermatitis in an amputee. Br J Dermatol. 1999 Aug. 141(2):380-1. [Medline].

  2. Sbano P, Miracco C, Risulo M, Fimiani M. Acroangiodermatitis (pseudo-Kaposi sarcoma) associated with verrucous hyperplasia induced by suction-socket lower limb prosthesis. J Cutan Pathol. 2005 Jul. 32(6):429-32. [Medline].

  3. Landthaler M, Langehenke H, Holzmann H, Braun-Falco O. [Mali's acroangiodermatitis (pseudo-Kaposi) in paralyzed legs]. Hautarzt. 1988 May. 39(5):304-7. [Medline].

  4. Kim TH, Kim KH, Kang JS, Kim JH, Hwang IY. Pseudo-Kaposi's sarcoma associated with acquired arteriovenous fistula. J Dermatol. 1997 Jan. 24(1):28-33. [Medline].

  5. Larralde M, Gonzalez V, Marietti R, Nussembaum D, Peirano M, Schroh R. Pseudo-Kaposi sarcoma with arteriovenous malformation. Pediatr Dermatol. 2001 Jul-Aug. 18(4):325-7. [Medline].

  6. Lyle WG, Given KS. Acroangiodermatitis (pseudo-Kaposi's sarcoma) associated with Klippel-Trenaunay syndrome. Ann Plast Surg. 1996 Dec. 37(6):654-6. [Medline].

  7. Zutt M, Emmert S, Moussa I, et al. Acroangiodermatitis Mali resulting from arteriovenous malformation: report of a case of Stewart-Bluefarb syndrome. Clin Exp Dermatol. 2008 Jan. 33(1):22-5. [Medline].

  8. Wei-Min Tan A, Lee JS, Pramono ZA, Chong WS. Acroangiodermatitis of Mali in protein C deficiency due to a novel PROC gene mutation. Am J Dermatopathol. 2012 Apr. 34(2):e19-21. [Medline].

  9. Horiguchi Y, Takahashi K, Tanizaki H, Miyachi Y. Case of bilateral acroangiodermatitis due to symmetrical arteriovenous fistulas of the soles. J Dermatol. 2015 Oct. 42 (10):989-91. [Medline].

  10. Kazakov DV, Sima R, Michal M. Hemosiderotic fibrohistiocytic lipomatous lesion: clinical correlation with venous stasis. Virchows Arch. 2005 Jul. 447(1):103-6. [Medline].

  11. Parsi K, O'Connor A, Bester L. Stewart-Bluefarb syndrome: Report of five cases and a review of literature. Phlebology. 2014 Aug 13. [Medline].

  12. Pimentel MI, Cuzzi T, de Azeredo-Coutinho RB, Vasconcellos Éde C, Benzi TS, de Carvalho LM. Acroangiodermatitis (pseudo-Kaposi sarcoma): a rarely-recognized condition. A case on the plantar aspect of the foot associated with chronic venous insufficiency. An Bras Dermatol. 2011 Jul-Aug. 86(4 Suppl 1):S13-6. [Medline].

  13. Kanitakis J, Narvaez D, Claudy A. Expression of the CD34 antigen distinguishes Kaposi's sarcoma from pseudo-Kaposi's sarcoma (acroangiodermatitis). Br J Dermatol. 1996 Jan. 134(1):44-6. [Medline].

  14. Pires A, Depairon M, Ricci C, Krayenbuhl B, Panizzon RG. Effect of compression therapy on a pseudo-Kaposi sarcoma. Dermatology. 1999. 198(4):439-41. [Medline].

  15. Orenay OM, Sarifakioglu E, Ozcan D, Köseoglu HK. An alternative treatment for acroangiodermatitis: intermittent pneumatic compression therapy. Acta Dermatovenerol Alp Panonica Adriat. 2013 Dec. 22(4):91-2. [Medline].

  16. Rashkovsky I, Gilead L, Schamroth J, Leibovici V. Acro-angiodermatitis: review of the literature and report of a case. Acta Derm Venereol. 1995 Nov. 75(6):475-8. [Medline].

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The physical findings in this patient who is HIV negative remained the same over a 3-year period.
Lesions on the shin of a patient who is HIV negative.
Acroangiodermatitis on histopathologic examination.
Example of acroangiodermatitis on histopathologic examination.
Higher-power view of acroangiodermatitis on histopathologic examination.
Classic Kaposi sarcoma on the foot of an elderly patient who is HIV negative. Compare this photo to the clinical photos of acroangiodermatitis.
Classic Kaposi sarcoma on histopathologic examination.
 
 
 
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