Pseudo-Kaposi Sarcoma (Acroangiodermatitis)

Pseudo-Kaposi Sarcoma (Acroangiodermatitis)

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Since its original description, acroangiodermatitis has been described in amputees (especially in those with poorly fitting suction-type devices), [1, 2] in patients with paralyzed legs, [3] in patients undergoing hemodialysis (from arteriovenous shunts distally), [4] and in association with hepatitis C. It has been documented in chronic venous insufficiency, in vascular malformations [5] (eg, Klippel-Trenaunay syndrome, [6] Stewart-Bluefarb syndrome, [7]  Prader-Labhart-Willi syndrome), protein-C deficiency, [8] and due to symmetrical arteriovenous fistulae. [9]

Acroangiodermatitis is a hyperplasia of preexisting vasculature, as opposed to Kaposi sarcoma, in which the vascular proliferation is independent of the existing vessels. It is usually seen as a complication of severe chronic venous stasis (hypostasis and elevated venous pressure) of the lower legs and the feet. Conversely, though less common, congenital or acquired arteriovenous anomalies can result in high venous pressure. Acroangiodermatitis can occur in amputees of the lower extremity.

Severe chronic venous stasis and the insufficiency of the calf muscle pump result in an elevated capillary pressure. Plethysmographic studies demonstrate the insufficiency of both the muscular pump of the calf and the venous pump of the foot in acroangiodermatitis.

The lack of the muscle pump and the disturbed innervation of vessels both may be of pathogenetic importance in paralyzed extremities. Others suggest that paralysis could generate the cutaneous lesions by increasing venous stasis and enhancing arteriovenous channels. In Klippel-Trenaunay syndrome, a high perfusion rate and a high oxygen saturation may be involved in the development of the lesions.

Acroangiodermatitis can occur in cases of acquired iatrogenic arteriovenous fistula from hemodialysis. Some cases have been reported that may resolve after thrombosis or surgical elimination of the shunt.

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).

Fewer than 100 cases have been reported. It is probably uncommon but not rare. A tendency to not report additional cases that do not provide any new information to the literature is likely.

No exact data are available.

The condition is more frequent in males than in females.

Most cases have been described in adults.

Individual lesions may persist unchanged for several years. The lesions of acroangiodermatitis can ulcerate and bleed and are at risk of infection. Differentiate developmental arteriovenous malformations of the feet from acroangiodermatitis because the prognosis for the former is more severe. Mortality and morbidity depend on the underlying condition.

Educate patients about skin care and avoiding mechanical trauma that could induce ulceration or bleeding. Recommend that patients reduce their body weight in appropriate cases.

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

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].

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].

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].

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].

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

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].

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].

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].

Huguen J, Bonsang B, Lemasson G, Misery L, Brenaut E. Image Gallery: Acroangiodermatitis or pseudo-Kaposi sarcoma: two cases in patients with paralyzed legs. Br J Dermatol. 2016 Jun. 174 (6):e84. [Medline].

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

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

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].

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].

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].

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].

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].

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.

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 Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, American Society of Dermatopathology, Association of Professors of Dermatology, Society for Investigative Dermatology, Texas Dermatological Society

Disclosure: Book royalties from Elsevier publishers.

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

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.

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.

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.

Pseudo-Kaposi Sarcoma (Acroangiodermatitis)

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