Schamberg's Disease Progressive Pigmented Purpuric Dermatitis

Schamberg's Disease

I. Definition:

Schamberg's disease, or progressive pigmented purpuric dermatitis, is a chronic discoloration of the skin which usually affects the legs and often spreads slowly. This disease is more common in males and may occur at any age from childhood onward.

II. Cause:

People who develop Schamberg's disease have leaky blood vessel walls which allow red blood cells to slip through into the skin. The red cells in the skin then fall apart and release their iron which has a rust color. This accounts for the orange tint of the rash.

III. Symptoms:

The lesions are most frequent on the lower limbs, but may occur anywhere on the body, including the hands. They may vary in number.
They consist of irregular patches of orange or brown pigmentation due to hemosiderin (iron), with characteristic "cayenne pepper" spots appearing within and at the edge of old lesions.
There are usually no symptoms, although there may be some slight itching.
The eruption may persist for many years. The pattern of the eruption changes, with slow extension and often some clearing of the original lesions.

IV. Treatment:

There is no permanent cure for Schamberg's disease; however, the itching can be controlled by a cortisone cream.

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