The condition is caused by inflammation of capillaries near the surface of skin and subsequent leaking of red blood cells into surrounding tissues.
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 consist of irregular patches of orange or brown pigmentation with characteristic "cayenne pepper" spots appearing within and at the edge of old lesions.
Schamberg's disease, or progressive pigmented purpuric dermatosis, is a chronic discoloration of the skin which usually affects the legs and often spreads slowly.
[4] Although the underlying cause for the leaky blood vessels is almost always unknown, researchers suggest some potential triggers.
[5] These include the body's inflammatory reaction to some agent, such as a viral infection or a prescription or over the counter medication or supplement, such as thiamine and aspirin.
[7] With a complete history, the results from visual examination, and the aid of appropriate laboratory testing, a dermatologist can usually determine whether the skin lesions are in fact due Schamberg's disease.
While reviewing medical history is important to diagnose this condition, it is essential that the skin be physically examined.
[7] This is because some people have been observed to be sensitive to these agents, and the body initiates an inflammatory reaction if exposed to them again, which causes further capillary inflammation and red blood cell leakage.
[7] Several research studies have indicated that Schamberg's disease can be controlled and the number of lesions can be reduced with the use of a drug called aminaphtone.
[11] A study published in 2014 on the Journal of the German Society of Dermatology (Deutsche Dermatologische Gesellschaft) concludes that oral rutoside and ascorbic acid may be an efficient and well tolerated treatment for PPPD, with a recommendation for early treatment for best clinical outcome.
A case series published in 2012 describes the treatment of five patients with Schamberg's disease of the lower extremities using Advanced Fluorescence Technology (AFT) pulsed light with favorable results.
[3] Skin lesions may cause psychological discomfort, where patients may require reassurance to help with stress and anxiety.