Cutaneous Vascular Diseases

Abstract

There are four closely related dermatoses, associated with purpuric manifestations, which primarily affect the legs and all produce punctate pigmented macules. They exhibit increased capillary permeability, or fragility. These conditions are:—

• 1. Progressive Pigmentary Dermatosis (Schamberg’s disease) This is an idiopathic, purpuric, asymptomatic, slowly progressive, pigmentary disorder of the skin occurring principally in males. The initial lesions are the so-called “cayenne-pepper spots” brownish-reddish puncta which tend to coalesce and form diffusely pigmented patches. It is a type of stasis dermatitis commencing on the ankles; the patches enlarge and spread upwards along the lower extremities.

• 2. Purpura Annularis Telangiectodes (Majocchi’s disease) This rare disease is characterised by telangiectatic, purpuric and atrophic lesions which occur usually on the lower extremities. Four stages occur, namely:—(1) telangiectatic, (2) purpuric, (3) pigmentary and (4) atrophic. The early lesions are minute bright red puncta. Discoid and circulate patches slowly increase in size and, by spreading peripherally with central atrophic healing, they form annular lesions. The disease usually clears up spontaneously after several months.

• 3. Pigmented Purpuric Lichenoid Dermatitis (Gougerot —Blum) This is a chronic superficial dermatosis of insidious onset. The primary lesions are minute lichenoid, red to reddish-brown, papules which are sometimes haemorrhagic and tend to fuse into elevated plaques with irregular boundaries. The legs, thighs, lower trunk and arms are the favourite sites. It may simulate lichen planus.

• (4) Angioma Serpiginosum (Hutchinson) A rare naevoid type of telangiectasia, forming an irregular vascular network. It is an idiopathic purpuric eruption characterised by the formation of puncta in small rings. These puncta are bright-red and occur in groups which extend peripherally and clear centrally; this results in the formation of small rings or serpiginous lines. Purpura is said to be absent. The eruption is symmetrical and most commonly affects the legs.