Stewart's bluefarb syndromeI87.2

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 03.08.2021

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Synonym(s)

Blue Color Stewart Syndrome; Pseudoangiosarcomatosis; pseudocaposi sarcoma (Stewart-Bluefarb-type); pseudo-Kaposi's sarcoma

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HistoryThis section has been translated automatically.

Bluefarb & Adams, 1967; Stewart, 1967

DefinitionThis section has been translated automatically.

Pseudo-Kaposi sarcoma occurring in arteriovenous shunts (e.g. in Parkes-Weber syndrome), i.e. clinically reminiscent of a Kaposi sarcoma, benign reactive skin change characterized by hyperplasia of small vessels with accompanying fibrosis.

ManifestationThis section has been translated automatically.

Mostly occurring at a younger age

LocalizationThis section has been translated automatically.

Located mainly in the area of the lower legs and back of the foot. Mostly occurring on one side.

Clinical featuresThis section has been translated automatically.

Sharply defined, plaque-like, reddish-brown to livid, spots (patches). These gradually develop into plaques and nodules. Usually rapid progression. In addition, pain, edema, hypertrophy of the limb, varicose veins, and hyperthermia may be present as signs of an AV shunt.

HistologyThis section has been translated automatically.

Proliferation of fibroblasts and vessels; evidence of erythrocyte extravasation, hemosiderin deposits, spindle cells and atypia. In some cases there are histologically visible AV connections.

DiagnosisThis section has been translated automatically.

Arteriovenous fistula.

Differential diagnosisThis section has been translated automatically.

Acroangiodermatitis (Mali type): by definition, Mali type acroangiodermatitis is due to chronic venous insufficiency.

Congestive dermatitis in CVI: evidence of CVI.

Kaposi's sarcoma. Histology is diagnostic. No evidence of vascular disease. Immune suppression in endemic Kaposi's sarcoma.

TherapyThis section has been translated automatically.

Operative removal of the shunt by surgeons.

LiteratureThis section has been translated automatically.

  1. Bluefarb SM, Adams LA (1967) Arteriovenous malformation with angiodermatitis. Arch Dermatol 96: 176-181
  2. Larralde M et al (2001) Pseudo-Kaposi sarcoma with arteriovenous malformation. Pediatr Dermatol 18: 325-327
  3. Stewart WM (1967) Fausse angiosarcomatose de Kaposi par fistules arterioveineuses multiples. Bull Soc Fr Derm Syph 74: 664-665

  4. Silvestre Torner N et al (2020) Stewart-Bluefarb syndrome associated with an iatrogenic arteriovenous fistula. Nefrologia (Engl Ed) S0211-6995(20)30180-6.

  5. Ueki H et al (1986) Stewart-Bluefarb syndrome (caposiform arterio-venous short circuit fistula with bone changes). Dermatologist 37: 673-675
  6. Utermann S et al (2000) Successful long-term therapy of Stewart-Bluefarb syndrome. Dermatologist 51: 336-339

Authors

Last updated on: 03.08.2021

Author: Prof. Dr. med. Peter Altmeyer