Acroangiodermatitis of mali: A rare vascular phenomenon

Abstract

Acroangiodermatitis (synonym pseudo-Kaposi sarcoma) is an unusual, benign condition which clinically presents as purple-colored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities in patients with chronic venous insufficiency and arteriovenous malformations. It resembles aggressive conditions like Kaposi's sarcoma and requires histopathological examination for its diagnosis. We report two such cases of acroangiodermatitis. Histopathology of both the cases showed dilated capillaries in the dermis with extravasated red blood corpuscles (RBCs), hemosiderin deposits, and hyperplastic granulation tissue. Both were treated with oral antibiotics and topical steroids. The ulcers showed a good response within 2 months of treatment.

Acroangiodermatitis (Pseudo-Kaposi sarcoma)

“…It is a proliferation of pre-existing vasculature seen in venous hypertension, arteriovenous malformation, or acquired iatrogenic arteriovenous (AV) fistula. [1] Most of the previously reported cases were associated with venous insufficiency, but few cases of spontaneous presentation have also been reported. [3] It is rarely reported in hereditary coagulation defects, i.e., carrier of the thrombophilic 20210A mutation in the prothrombin gene and homozygous activated protein C resistance.…”

“…[4] Various variants of acroangiodermatitis have been described including Stewart-Bluefarb syndrome (congenital arteriovenous malformation of the lower leg with multiple AV shunts), Mali type (an exaggerated stasis dermatitis seen in elderly patients, which is usually bilateral associated with chronic venous insufficiency), Acroangiodermatitis in first pregnancy presenting as gravity purpura (Dermite ocre of Favre) on lower legs over site of venous varicosities, Angiodermatitis occurring after placement of the arteriovenous shunt for hemodialysis in chronic renal failure. [15]…”

“…[7] Underlying vascular pathology like arterio-venous malformations should be surgically managed with elimination of the shunts and embolization for small fistulae. [15]…”

“…[1] It is a benign condition which clinically presents as violaceous macules, indurated plaques or nodules usually bilaterally on the extensor surfaces of lower extremities. The term acroangiodermatitis of Mali was introduced for the first time by Mali et al .…”

Pseudokaposi′s sarcoma

“…The most important is the correction of the underlying pathology with compression stockings and surgery for varicose veins. Other treatment modalities tried with good response are topical steroid therapy,[9] oral erythromycin,[9] and dapsone. [8] Our case also showed a good response to oral dapsone.…”