Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis

Abstract

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.

Dusky erythematous plaques over the lower leg associated with bony deformity

“…[ 1 ] PWS includes a triad of high-flow arteriovenous malformation with a capillary or venous malformation and associated limb hypertrophy. [ 2 ] The severity of the associated angiodermatitis secondary to arteriovenous malformation is graded as Stage I: asymptomatic macules and plaques; stage II: pulsatile expansile lesions; stage III: destructive stage with complications like skin ulceration and lytic changes in underlying bones; and stage IV: overt heart failure. [ 3 ] The present patient had stage III involvement.…”

“…Conservative measures include protection from local trauma, rest, compression stockings, and regular cardiac monitoring. [ 1 2 ]…”