Stewart-Bluefarb Syndrome

Hueso, L.; Llombart, B.; Alfaro‐Rubio, A.; Serra‐Guillén, C.; Requena, Celia; González, M.; Cano, B.; Nagore, Eduardo; Sanmartín, O.; Botella‐Estrada, Rafael; Guillén, C.

doi:10.1016/s1578-2190(07)70510-3

Cited by 5 publications

(12 citation statements)

References 18 publications

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“…SBS presents as violaceous papuloplaque or nodular lesions with presence of varicose veins, palpable thrill, bruit on auscultation, raised local temperature, enlargement of the limb along with capillary haemangiomas and other manifestations of AVM. Clinically, SBS is usually unilateral and the histopathological changes are more in the deeper dermis in contrast to acroangiodermatitis of Mali which may be bilateral and histopathological changes are confined to the superficial dermis 8…”

Section: Discussionmentioning

confidence: 99%

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Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis

et al. 2019

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Parkes Weber syndrome (PWS) is a rare disorder characterised by arteriovenous (AV) fistula, along with capillary, lymphatic, venous malformations and limb hypertrophy. Stewart-Bluefarb syndrome is a variant of acroangiodermatitis, which is associated with congenital AV malformation/fistulas. It usually begins early in life, unilaterally over lower extremities presenting as violaceous to dusky coloured macules, papules or plaques with tendency to ulcerate. We are reporting a case of AV malformation fulfilling the triad of PWS and presenting with acroangiodermatitis.

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Section: Discussionmentioning

confidence: 99%

“…Diagnosis is done by Doppler ultrasonography of the affected limb, a non-invasive and highly sensitive screening method. Arteriography, with the angiographic hallmark of early venous filling proportional to the extent of AV connections, is the gold standard investigation 3 8…”

Section: Discussionmentioning

confidence: 99%

Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis

et al. 2019

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“…Verrucous lesions, ulceration, edema, soft tissue hypertrophy, varicose veins, stasis changes, and hypertrichosis may also be present. 1 Histopathologically, it is characterized by an often exuberant proliferation of multiple thick-walled round vessels in a sclerotic and edematous dermis. The vessels have a fairly regular distribution and shape and are mainly situated on the papillary dermis.…”

Section: Discussionmentioning

confidence: 99%

“…Surgery should be considered in cases involving functional impotence, refractory pain, recurrent infection, bleeding, or heart failure. 1…”

Section: Discussionmentioning

confidence: 99%

Progressive erythematous plaques in a young man

2015

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“…4 Lower limb lesions typically occur on the extensor surfaces and in particular the dorsal aspects of the foot, ankle or calf. 1–4,6…”

Section: Introductionmentioning

confidence: 99%

Stewart–Bluefarb syndrome: Report of five cases and a review of literature

2014

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Stewart-Bluefarb syndrome is a rare angioproliferative disorder characterised by acroangiodermatitis associated with an underlying arteriovenous shunt. This condition should be differentiated from acroangiodermatitis of Mali classically described in association with chronic venous insufficiency. Patients with Stewart-Bluefarb syndrome typically present with lower leg pigmented macules, papules and plaques that can coalesce to form larger confluent patches of pigmentation. Recognition of Stewart-Bluefarb syndrome may be difficult or delayed as the cutaneous manifestations may resemble a variety of other dermatological conditions. Most commonly, acroangiodermatitis may be confused with Kaposi's sarcoma and the condition is often referred to as 'Pseudo-Kaposi's sarcoma'. Acroangiodermatitis may also resemble or coexist with pigmentation of chronic venous insufficiency. As seen in this report, acroangiodermatitis may also be clinically confused with the 'cavernous' form of a capillary malformation. Here, we describe five patients with Stewart-Bluefarb syndrome. In one female and two male patients the diagnosis was delayed as the acroangiodermatitis closely resembled other conditions. All underlying arterio-venous communications were initially diagnosed on duplex ultrasound and confirmed with magnetic resonance angiography. Four patients were found to have a congenital arterio-venous malformation while one was diagnosed with a post-thrombotic arterio-venous fistula. Management included observation and intervention using a variety of techniques including percutaneous or trans-catheter embolisation, endovenous laser, radiofrequency ablation and foam ultrasound guided sclerotherapy. This case series highlights the challenges involved in the diagnosis and management of Stewart-Bluefarb syndrome. Given the local and systemic sequelae of high flow shunts, correct diagnosis and early detection of the underlying arterio-venous abnormality is crucial in the long-term management of these patients and in preventing the associated complications.

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Stewart-Bluefarb Syndrome

Cited by 5 publications

References 18 publications

Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis

Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis

Progressive erythematous plaques in a young man

Stewart–Bluefarb syndrome: Report of five cases and a review of literature

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