CAPILLARITIS or PROGRESSIVE PIGMENTING PURPURA

By Gary M. White, MD

Capillaritis or Progressive Pigmented Purpura


The term capillaritis (aka progressive pigmenting purpura (PPP), pigmentary purpuric eruptions, progressive pigmentary dermatosis, progressive pigmenting purpura, purpura pigmentosa chronica) refers to a group of diseases characterized by red, purple or brown color changes in the skin related to leakage of blood out of capillaries.

Reported Triggers of Capillaritis

Clinical

Tiny purpuric macules coalescent into plaques is typical. The lower legs are the most common site (in one study 80%). Various forms including annular and pigmented occur.

Treatment

Usually, no treatment is effective, but if there is an obvious trigger, it should be avoided. For example, if eczema is present or proceeds the lesions, the use topical steroids/emolliation should be recommended. If the lesions are on the legs in areas of swelling, support hose may be tried. But usually, there is no good treatment and the patient should merely be reassured. Narrow band UVB has been effective in various case reports. A trial of a potent topical steroid for 6 weeks is a reasonable option, but usually is unrewarding.

In a retrospective review of 35 patients with PPP treated with 2 x 50 mg BID rutoside and 1,000 mg ascorbic acid daily [J Dtsch Dermatol Ges. 2014;12:1112-9], 71.4% experienced complete clearance and 20.0% an improvement of more than 50%. Nine participants (25.1%) relapsed after discontinuation. In seven, rutoside and ascorbic acid was re-initiated, and all responded again. Only three participants reported mild adverse effects. The mean treatment duration was 8.2 months. In one uncontrolled study of 3 patients, oral rutoside (50 mg BID) and ascorbic acid (500 mg BID) was very effective [JAAD 1999;41;207]. At the end of the 4-week treatment period, complete clearance of the skin lesions was achieved in all 3 patients.

Additional Photos

Capillaritis or Progressive Pigmented Purpura Capillaritis or Progressive Pigmented Purpura

A solitary lesion on the ankle, a typical location.
Capillaritis or Progressive Pigmented Purpura

8 year old with pigmentation in a dermatomal pattern.
Capillaritis or Progressive Pigmented Purpura

A good example of the reason behind the term "progressive pigmented purpura".
Capillaritis or Progressive Pigmented Purpura

Cayenne pepper red and brown.
Capillaritis or Progressive Pigmented Purpura

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