Case Reports

Acroangiodermatitis of Mali and Stewart-Bluefarb Syndrome

Cutis. 2019 June;103(06):336-339

References

Parsi K, O’Connor AA, Bester L. Stewart-Bluefarb syndrome: report of five cases and a review of literature. Phlebology. 2015;30:505-514.
Larralde M, Gonzalez V, Marietti R, et al. Pseudo-Kaposi sarcoma with arteriovenous malformation. Pediatr Dermatol. 2001;18:325-327.
Nakanishi G, Tachibana T, Soga H, et al. Pseudo-Kaposi’s sarcoma of the hand associated with acquired iatrogenic arteriovenous fistula. Indian J Dermatol. 2014;59:415-416.
Landthaler M, Langehenke H, Holzmann H, et al. Mali’s acroangiodermatitis (pseudo-Kaposi) in paralyzed legs. Hautarzt. 1988;39:304-307.
Trindade F, Requena L. Pseudo-Kaposi’s sarcoma because of suction socket lower limb prosthesis. J Cutan Pathol. 2009;36:482-485.
Yu-Lu W, Tao Q, Hong-Zhong J, et al. Non-tender pedal plaques and nodules: pseudo-Kaposi’s sarcoma (Stewart-Bluefarb type) induced by trauma. J Dtsch Dermatol Ges. 2015;13:927-930.
Del-Río E, Aguilar A, Ambrojo P, et al. Pseudo-Kaposi sarcoma induced by minor trauma in a patient with Klippel-Trenaunay-Weber syndrome. Clin Exp Dermatol. 1993;18:151-153.
Archie M, Khademi S, Aungst D, et al. A rare case of acroangiodermatitis associated with a congenital arteriovenous malformation (Stewart-Bluefarb Syndrome) in a young veteran: case report and review of the literature. Ann Vasc Surg. 2015;29:1448.e5-1448.e10.
Mali JW, Kuiper JP, Hamers AA. Acro-angiodermatitis of the foot. Arch Dermatol. 1965;92:515-518.
Bluefarb SM, Adams LA. Arteriovenous malformation with angiodermatitis. stasis dermatitis simulating Kaposi’s disease. Arch Dermatol. 1967;96:176-181.
Earhart RN, Aeling JA, Nuss DD, et al. Pseudo-Kaposi sarcoma. A patient with arteriovenous malformation and skin lesions simulating Kaposi sarcoma. Arch Dermatol. 1974;110:907-910.
Lugovic´ L, Pusic´ J, Situm M, et al. Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports. Acta Dermatovenerol Croat. 2007;15:152-157.
Horiguchi Y, Takahashi K, Tanizaki H, et al. Case of bilateral acroangiodermatitis due to symmetrical arteriovenous fistulas of the soles. J Dermatol. 2015;42:989-991.
Dog˘an S, Boztepe G, Karaduman A. Pseudo-Kaposi sarcoma: a challenging vascular phenomenon. Dermatol Online J. 2007;13:22.
Mazloom SE, Stallings A, Kyei A. Differentiating intralymphatic histiocytosis, intravascular histiocytosis, and subtypes of reactive angioendotheliomatosis: review of clinical and histologic features of all cases reported to date. Am J Dermatopathol. 2017;39:33-39.
Rongioletti F, Rebora A. Cutaneous reactive angiomatoses: patterns and classification of reactive vascular proliferation. J Am Acad Dermatol. 2003;49:887-896.
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;134:44-46.
Pires A, Depairon M, Ricci C, et al. Effect of compression therapy on a pseudo-Kaposi sarcoma. Dermatology. 1999;198:439-441.
Hayek S, Atiyeh B, Zgheib E. Stewart-Bluefarb syndrome: review of the literature and case report of chronic ulcer treatment with heparan sulphate (Cacipliq20^®). Int Wound J. 2015;12:169-172.
Varyani N, Thukral A, Kumar N, et al. Nonhealing ulcer: acroangiodermatitis of Mali. Case Rep Dermatol Med. 2011;2011:909383.
Mehta AA, Pereira RR, Nayak C, et al. Acroangiodermatitis of Mali: a rare vascular phenomenon. Indian J Dermatol Venereol Leprol. 2010;76:553-556.
Rashkovsky I, Gilead L, Schamroth J, et al. Acro-angiodermatitis: review of the literature and report of a case. Acta Derm Venereol. 1995;75:475-478.

Comment

Presentation of AAD
Acroangiodermatitis is a rare chronic inflammatory skin process involving a reactive proliferation of capillaries and fibrosis of the skin that resembles Kaposi sarcoma both clinically and histopathologically. The condition has been reported in patients with chronic venous insufficiency,¹ congenital arteriovenous malformation,² acquired iatrogenic arteriovenous fistula,³ paralyzed extremity,⁴ suction socket lower limb prosthesis (amputees),⁵ and minor trauma.^6-8 The lesions of AAD tend to be circumscribed, slowly evolving, red-violaceous (or brown or dusky) macules, papules, or plaques that may become verrucous or develop into painful ulcerations. They generally occur on the distal dorsal aspects of the lower legs and feet.¹¹⁰

Variants of AAD
Mali et al⁹ first reported cutaneous manifestations resembling Kaposi sarcoma in 18 patients with chronic venous insufficiency in 1965. Two years later, Bluefarb and Adams¹⁰ described kaposiform skin lesions in one patient with a congenital arteriovenous malformation without chronic venous insufficiency. It was not until 1974, however, that Earhart et al¹¹ proposed the term pseudo-Kaposi sarcoma.^10,11 Based on these findings, AAD is described as 2 variants: Mali type and SBS.

Mali-type AAD is more common and typically occurs in elderly men. It classically presents bilaterally on the lower extremities in association with severe chronic venous insufficiency.⁵ Skin lesions usually occur on the medial aspect of the lower legs (as in patient 1), dorsum of the heel, hallux, or second toe.¹²

The etiology of Mali-type AAD is poorly understood. The leading theory is that the condition involves reduced perfusion due to chronic edema, resulting in neovascularization, fibroblast proliferation, hypertrophy, and inflammatory skin changes. When AAD occurs in the setting of a suction socket prosthesis, the negative pressure of the stump-socket environment is thought to alter local circulation, leading to proliferation of small blood vessels.^5,13

Stewart-Bluefarb syndrome usually involves a single extremity in young adults with congenital arteriovenous malformations, amputees, and individuals with hemiplegia or iatrogenic arteriovenous fistulae (as in patient 2).¹ It was once thought to occur secondary to Klippel-Trenaunay-Weber syndrome; however, SBS rarely is accompanied by limb hypertrophy.⁹ Pathogenesis is thought to involve an angiogenic response to a high perfusion rate and high oxygen saturation, which leads to fibroblast proliferation and reactive endothelial hyperplasia.^1,14

Diagnosis and Differential Diagnosis
Prompt identification of an underlying arteriovenous anomaly is critical, given the sequelae of high-flow shunts, which may result in skin ulceration, limb length discrepancy, cortical thinning of bone with regional osteoporosis, and congestive heart failure.^1,5 Duplex ultrasonography is the first-line diagnostic modality because it is noninvasive and widely available. The key doppler feature of an arteriovenous malformation is low resistance and high diastolic pulsatile flow,¹ which should be confirmed with magnetic resonance angiography or computed tomography angiography if present on ultrasonography.

The differential diagnosis of AAD includes Kaposi sarcoma, reactive angioendotheliomatosis, diffuse dermal angiomatosis, intravascular histiocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis.^15,16 These entities present as multiple erythematous, violaceous, purpuric patches and plaques generally on the extremities but can have a widely varied distribution. Some lesions evolve to necrosis or ulceration. Histopathologic analysis is useful to differentiate these entities.

Histopathology
The histopathologic features of AAD can be nonspecific; clinicopathologic correlation often is necessary to establish the diagnosis. Features include a proliferation of small thick-walled vessels, often in a lobular arrangement, in an edematous papillary dermis. Small thrombi may be observed. There may be increased fibroblasts; plump endothelial cells; a superficial mixed infiltrate comprised of lymphocytes, histiocytes, and eosinophils; and deposition of hemosiderin.^2,5 These characteristics overlap with features of Kaposi sarcoma; AAD, however, lacks slitlike vascular spaces, perivascular CD34⁺expression, and nuclear atypia. A negative HHV-8 stain will assist in ruling out Kaposi sarcoma.^1,17

Management
Treatment reports are anecdotal. The goal is to correct underlying venous hypertension. Conservative measures with compression garments, intermittent pneumatic compression, and limb elevation are first line.¹⁸ Oral antibiotics and local wound care with topical emollients and corticosteroids have been shown to be effective treatments.^19-21

Oral erythromycin 500 mg 4 times daily for 3 weeks and clobetasol propionate cream 0.05% healed a lower extremity ulcer in a patient with Mali-type AAD.²¹ In another patient, conservative treatment of Mali-type AAD failed, but rapid improvement of 2 lower extremity ulcers resulted after 3 weeks of oral dapsone 50 mg twice daily.²²

A tissue matrix–protective agent (a heparan sulfate mimetic) was reported to completely resolve a patient’s lower extremity ulcer secondary to SBS after other treatment modalities failed.¹⁹ In the SBS variant of AAD, treatment should be directed toward obliterating the underlying arteriovenous malformation, which can be achieved by selective embolization, endovenous ablation, sclerotherapy, or surgical intervention.^1,2

Conclusion

Acroangiodermatitis is a rare entity that is characterized by erythematous violaceous papules and plaques of the extremities, commonly in the setting of chronic venous insufficiency or an arteriovenous shunt. Histopathologic analysis shows proliferation of capillaries with fibrosis, extravasation of erythrocytes, and deposition of hemosiderin without the spindle cells and slitlike vascular spaces characteristic of Kaposi sarcoma. Detection of an underlying arteriovenous malformation is essential, as the disease can have local and systemic consequences, such as skin ulceration and congestive heart failure.¹ Treatment options are conservative, directed toward local wound care, compression, and management of complications, such as ulceration and infection, as well as obliterating any underlying arteriovenous malformation.

Lambert-Eaton Myasthenic Syndrome and Merkel Cell Carcinoma

Acroangiodermatitis of Mali and Stewart-Bluefarb Syndrome

References

Comment

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