Pigmented purpuric dermatosis (PPD) is a rare condition. It causes red, purple or brown stains on the skin, particularly the lower legs.
Therefore, is very important for vein specialists to know about this condition. Pigmented purpuric dermatosis can easily be mistaken for skin damage secondary to varicose veins. Such skin damage is venous eczema (red) or haemosiderin (brown) skin damage secondary to varicose veins.
This occurs because the varicose veins, or hidden varicose veins, have been left untreated for too long. This results in chronic inflammation around the ankles. This chronic inflammation starts damaging the skin causing the discolouration. In the CEAP classification, this is CEAP C4.
However, patients with pigmented purpuric dermatosis do not have any underlying varicose veins. In these patients, the little blood vessels called capillaries are “leaky”. This leads to red blood cells getting outside of the capillaries under the skin.
Initially, this causes a red or purple discolouration. As the blood breaks down, it goes a golden brown or brown colour. At this stage, it often looks like CEAP C4 skin damage.
If you have red, purple or brown stains around the ankles, you need to see a venous specialist. This is true even if you can’t see any varicose veins on your legs. You may well have “hidden varicose veins”.
The chances are that you have got varicose veins or hidden varicose veins causing inflammation of the skin. Failure to treat the underlying varicose veins or hidden varicose veins results in vein disease worsening. In CEAP C4 disease, approximately 1 in 20 people deteriorating to venous leg ulcers every year.
The most important test is a venous duplex ultrasound scan. This needs to be performed by a specialist vascular technologist. Because our vascular technologists use The Whiteley Protocol, they check the main deep and superficial veins of the legs. Following that, they also check for incompetent perforating veins. Patients with severe skin changes at the ankle often have incompetent perforating veins. Hence a quick scan performed by a doctor often misses this problem.
If incompetent varicose veins, hidden varicose veins or incompetent perforating veins are found, these can then be treated by The Whiteley Protocol.
However, in patients with pigmented purpuric dermatosis, the scan will often appear normal. The main veins in the legs will be competent. However, using the high definition of venous duplex ultrasound, it often is possible to see the very small veins underlying the skin discolouration. This is why we always use high definition duplex ultrasound at The Whiteley Clinic.
An additional factor is that in many patients, a small amount of fluid can be seen in the tissue. We call this “oedema”. When a scan shows oedema in the legs, it means that the veins are probably leaking.
In the case of a normal duplex ultrasound scan, it is clear that the skin discolouration isn’t due to underlying varicose veins. Therefore, we need a definitive test to make a diagnosis. In pigmented purpuric dermatosis, this definitive test is a skin biopsy.
A very small section of skin is removed using a punch biopsy. Expert histopathologists can then look at the skin and underlying capillaries to confirm whether pigmented purpuric dermatosis is present.
Most cases are “idiopathic”. This means that in most cases we do not know what the causes. There does not seem to be any link with any major underlying disease or problem. It is not related to any clotting problems or other serious health concerns in most patients. Therefore, there is rarely an underlying problem to fix.
In some patients, the pigmented purpuric dermatosis can continue to progress. In this case, it is called “Schamberg Disease“.
There are some other conditions that look like pigmented purpuric dermatosis. As such the same doctor should check the patient regularly. In this way a doctor monitors any changes. Also, this is another reason why making sure a venous duplex ultrasound scan and specialist histology has been performed in all cases.
In most cases, it is safe to leave pigmented purpuric dermatosis alone. However many patients want relief from either symptoms (aching or swelling). Others do not like the look of the veins.
The simplest treatment with very few risks is to wear compression stockings. In addition, bioflavonoid tablets with vitamin C can help.
Some people wish to be more aggressive with treatment. Such treatments are available including using steroids. For the cosmetic look, microsclerotherapy. However, this is quite an undertaking and may not give a good result.
Therefore, such treatments are only suitable for patients determined to take risks. Hence, a good relationship between the doctor and patient will ensure that treatment is closely monitored. In this way, any signs of complications or worsening can be acted upon quickly.
For more information about pigmented purpuric dermatosis see:
Tolaymat L; Hall MR. Dermatitis, Pigmented Purpuric. Treasure Island (FL): StatPearls Publishing; 2018 Jan-. Lots working