Progressive Pigmentary Purpura

Progressive pigmentary purpura is a skin disease that usually affects young people. It is also known as Schamberg's disease. The skin lesions can appear anywhere on the body but are usually found on the lower part of the legs, as well as the hands. The skin lesions have a high content of iron, which gives them an orange or brown color. Due to the irregular shape, they can be confused with more dangerous skin conditions and a biopsy is sometimes needed for a proper diagnostic, after the tissue is examined with a microscope.

In some cases, the skin lesions can have spots located either in the middle or around their edges. These are popularly known as cayenne pepper spots and usually do not cause any symptoms, although slight itching is possible. These skin lesions last for several years. Sometimes they can heal on their own without any treatment, or their size can slowly increase.

The lower limbs are most often affected by lesions but no area of the body is immune and they can be found anywhere. They usually look like blemishes or rashes with an orange or brown color. Most often found on the legs, the abrasions can be itchy, with an asymmetrical shape and spots that resemble cayenne pepper. It is possible for the spots to simply disappear on their own after only one or two weeks. In other cases, they persist for a long time.

The condition is also known as PPP. It is rare in kids and it usually starts in adulthood. For some reason, males have a higher risk than females to develop it. Progressive pigmentary purpura is considered to be a progressive one because it starts on the lower part of the legs, then covers a larger part of them and can eventually spread to other areas such as the palms.

Progressive pigmentary purpura is not a single disease but actually an umbrella term for a number of conditions with similar symptoms. Every person affected by it can have an unique problem. Depending on the case, it can last for several years, heal in one week or even return in cyclical outbreaks. The worst symptom of progressive pigmentary purpura is itching, so the cosmetic impact is more severe.

A doctor can diagnose progressive pigmentary purpura by performing a biopsy. Under a microscope, the small blood vessels in the skin are visibly surrounded by areas of inflammation. This damages their walls, allowing blood to leak out. It creates small red dots, which later change their color because the iron oxidates and turns into hemosiderin, a reaction similar to rusting.

What causes progressive pigmentary purpura?

Progressive pigmentary purpura happens if red cells infiltrate the skin as a result of damage to blood vessel walls. These cells have a high content of iron, which gives skin lesions their distinctive red or orange color. Progressive pigmentary purpura is a mysterious disease and doctors are not sure what exactly causes it. Some possible triggers include contact with compounds that increase sensitivity and viral infections. Drugs like aspirin and thiamine might also cause the condition.

In some cases, the trigger of progressive pigmentary purpura can be identified precisely. This can be a different skin disease or a reaction to medicine. Other causes are allergies to various products, such as chemical coloring agents, rubber, dyes used in clothing or food preservatives. Sometimes, the affected area is small and the cause is damaged blood vessels. These can be corrected through surgery.

Since a few situations when several generations of the same family have been affected are known, a genetic cause is also suspected. However, researchers have been unable to identify the exact gene or factor that can start progressive pigmentary purpura.

Treatment options

Progressive pigmentary purpura can't be cured or treated directly in any way. It rarely has any kind of symptoms, so treatment is rarely actually needed. Itching is the only possible issue. If this starts to cause trouble, antihistamine pills or cortisone creams can be used to cure it.

Modern medicine has attempted to find other treatment methods for progressive pigmentary purpura. Ultraviolet light therapy using narrow band radiation is one of the techniques that shows some promise. Aminaphtone, a medicine known to be effective against other problems with the veins, has been found to be useful in a separate study. It appears to limit the side effects of progressive pigmentary purpura, without curing the condition itself.

Some other treatments can reduce the intensity of symptoms, without any definitive proof validated by research. These include supporting the veins with a hose or ingesting supplements with vitamin C.

Progressive pigmentary purpura is pretty rare and has no symptoms except the visible skin lesions and sometimes itching. This makes progressive pigmentary purpura hard to diagnose and it is often mistaken for other diseases. Doctors might require a skin biopsy and extra blood tests in order to establish a definitive diagnosis. A clear result is needed because treatment can't be administered without it. This is because other diseases can cause very similar skin lesions but require completely different medication.

Pentoxifylline, commercially known as Trental, is an oral drug that can be prescribed in severe cases. It is now always effective and a cure lasts several months. It has no side effects besides a possible indigestion and it boosts blood circulation. Food supplements such as bioflavonoid complex with rutin or vitamin C are also said to be helpful.

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