Specialty dermatology ICD-9-CM 709.09 eMedicine derm/327 | ICD-10 L81.7 DiseasesDB 30753 Patient UK Schamberg disease | |
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Schamberg Disease, (also known as "Progressive pigmentary dermatosis of Schamberg", "Purpura pigmentosa progressiva" (PPP), and "Schamberg's purpura") is a chronic discoloration of the skin found in people of all ages, usually affecting the legs. It slowly spreads throughout the body, and is most common in males. It is named after Jay Frank Schamberg, who described it in 1901.
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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.
Cause
Schamberg's disease is caused by leaky blood vessels near the surface of the skin, capillaries, which allow red blood cells to slip through into the skin. The red blood cells in the skin then fall apart and release their iron which causes a rust color, this accounts for the orange tint of the rash.
Symptoms
The lesions are most frequent on the lower limbs, but may occur anywhere on the body, including the hands, arms, torso and even the neck. They may vary in number and erupt in mass numbers. They consist of irregular patches of orange or brown pigmentation due to the iron complex hemosiderin, 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.
Treatment
There is no cure for Schamberg's disease; however, the itching can be controlled by a cortisone cream, and Colchicine treatment has been successfully used to prevent recurrence of the symptoms.