title: 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.
abstract: Acroangiodermatitis (AD) is a rare angioproliferative disease manifesting with cutaneous lesions clinically similar to Kaposi's sarcoma. AD is a benign hyperplasia of preexisting vasculature and may be associated with acquired or congenital arteriovenous malformations (AVM), or severe chronic venous insufficiency (because of hypostasis, elevated venous pressure, arteriovenous shunting). Stewart-Bluefarb syndrome is the rare syndrome in which AD is associated with a congenital AVM. We present the case of a young veteran with a painful, chronic nonhealing ulcer and ipsilateral popliteal artery occlusion likely because of trauma, who elected transmetatarsal amputation for symptomatic relief. A 24-year-old male veteran presented with a 5-year history of a nonhealing dorsal left foot ulcer, resulting from a training exercise injury. He ultimately developed osteomyelitis requiring antibiotics, frequent debridements, multiple trials of unsuccessful skin substitute grafting, and severe unremitting pain. He noted a remote history of left digital deformities treated surgically as a child, and an AVM, previously endovascularly treated at an outside facility. Arterial duplex revealed somewhat dampened left popliteal, posterior tibial (PT), and dorsalis pedis (DP) artery signals with arterial brachial index of 1.0. CT angiography showed occlusion of the proximal to mid popliteal artery with significant calcifications felt initially to be a result of prior trauma. Pedal pulses were palpable and transcutaneous oxygen measurements revealed adequate oxygenation. Because of unremitting pain, the patient opted for amputation. Pathology revealed vascular proliferation consistent with AD. This case illustrates an unusual diagnosis of acroangiodermatitis, and a rare syndrome when associated with his underlying AVM (Stewart-Bluefarb syndrome). This resulted in a painful, chronic ulcer and was further complicated by trauma-related arterial occlusive disease. AD disease can hinder wound healing even in the presence of clinically evident blood flow. Although rare, such unusual diagnoses should be entertained particularly in the unusually young vascular surgical patient.
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA. Electronic address: mmarchie@mednet.ucla.edu.
authors.forename: Mark
authors.initials: M
authors.lastname: Archie
authors.affiliation: Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: Saieh
authors.initials: S
authors.lastname: Khademi
authors.affiliation: Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: David
authors.initials: D
authors.lastname: Aungst
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA; Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: Aksone
authors.initials: A
authors.lastname: Nouvong
authors.affiliation: Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: Shanna
authors.initials: S
authors.lastname: Freeman
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA; Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: Hugh
authors.initials: H
authors.lastname: Gelabert
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA; Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: David
authors.initials: D
authors.lastname: Rigberg
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA; Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA; Division of Vascular Surgery, Harbor-UCLA Medical Center, Los Angeles, CA.
authors.forename: Christian
authors.initials: C
authors.lastname: deVirgilio
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA.
authors.forename: Michael
authors.initials: M
authors.lastname: Lewis
authors.affiliation: Division of Vascular Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA; Greater Los Angeles Veterans Affairs Healthcare System, Surgical & Perioperative Careline, Los Angeles, CA.
authors.forename: Jessica
authors.initials: J
authors.lastname: O'Connell
mesh_terms: D000169:Acrodermatitis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D000671:Amputation, Surgical; D001157:Arterial Occlusive Diseases / Q000209:etiology / Q000601:surgery; D001165:Arteriovenous Malformations / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D001706:Biopsy; D002908:Chronic Disease; D016523:Foot Ulcer / Q000209:etiology / Q000601:surgery; D006801:Humans; D008297:Male; D011150:Popliteal Artery / Q000000981:diagnostic imaging / Q000293:injuries / Q000601:surgery; D012867:Skin / Q000098:blood supply*; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D057772:Vascular System Injuries / Q000150:complications / Q000601:surgery; D014728:Veterans; D014945:Wound Healing; D055815:Young Adult