Stewart's bluefarb syndrome I87.2

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.

Mostly occurring at a younger age

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

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.

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

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.

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