Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

What is Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)?

Schamberg disease is the most common type of a group of skin conditions called pigmented purpuric dermatoses (PPDs). These conditions tend to be long-term but harmless, and are marked by tiny reddish-purple spots, skin discoloration, and an increase in skin pigmentation—meaning that certain areas of the skin may appear brown, red, or yellow and patchy. The five types of PPDs include Schamberg’s purpura, Majocchi purpura, lichen aureus, Gougerot-Blum purpura, and eczematoid-like purpura of Doucas and Kapetanakis.

Schamberg disease is also known by several other names such as progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive, and Schamberg’s purpura. It is more commonly found in males and mainly affects the lower leg areas, but it can also show up on the thighs, buttocks, torso, or upper extremities.

What Causes Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)?

Schamberg disease is a condition where the precise cause is uncertain. It occurs when blood, particularly from small vessels known as capillaries, leaks into the skin, causing red blood cells to deposit into it. These red blood cells then release their iron from a protein called hemoglobin, responsible for carrying oxygen in the blood. This iron gives the skin a rust-like color, resulting in an orange or brown-colored rash.

We don’t exactly know why these capillaries become inflamed and start leaking, and there could be several contributing factors. These factors may include:

• Being in a position where your feet are below the level of your heart for a long time (gravitational dependency).
• Drinking alcohol.
• Taking certain medications, such as acetaminophen, aspirin, adalin, amlodipine, carbromal, chlordiazepoxide, glipizide, glybuzole, hydralazine, meprobamate, nitroglycerin, persantin, reserpine, thiamine, interferon-alfa, medroxyprogesterone acetate injections, topical fluorouracil, and sildenafil.
• Genetic factors, since the disease is sometimes seen in family members.

Some other factors that might lead to the disease include hepatitis B or malfunctioning of the immune system. Sometimes, it could also occur for no known reason at all (idiopathic).

Risk Factors and Frequency for Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

PPDs or pigmenting purpuric dermatoses comprise a range of vascular skin diseases that are relatively rare, making up only 0.18% of all reported skin diseases. The most common condition in this group is Schamberg’s Disease (SD).

• Schamberg’s Disease affects people of all races and ages, from 8 to 66, with an average age of 34. It has even been reported in a child as young as 19 months.
• It’s more common in males, with a male to female ratio between 5 and 3.8 to 1.
• While Schamberg’s Disease is more widespread in men, Purpura Annularis Telangiectodes of Majocchi is more prevalent in women.
• Lichen Aureus and Majocchi diseases typically develop in children or young adults.
• In contrast, the Gougerot and Blum dermatisis typically affects middle-aged and older men.

Signs and Symptoms of Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

Schamberg disease often doesn’t cause any discomfort but can sometimes lead to itching or pain. Interestingly, it’s a condition that can get better on its own. The disease is chronic and is usually seen in different stages simultaneously on a person’s body, meaning while new spots are forming, older ones are fading.

The spots caused by this disease are unique. They start as small, well-defined patches that can range in color from red to orange, brown, or even yellow. These patches often look like little specks of cayenne pepper and can vary greatly in shape and size. It’s common to first see these spots on the lower legs, but they can gradually spread to the rest of the body. The most frequently affected areas are both lower legs (usually on both sides), thighs, and buttocks. In rarer cases, the spots can appear on the upper body or be widespread on the entire body.

Testing for Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

Usually, doctors can diagnose this condition with a simple physical examination and by looking at the appearance of skin lesions. Sometimes, they may do a biopsy, which involves taking a small sample of skin to examine more closely. This can help rule out other potential diagnoses.

Doctors might also do a test called the Hess’s or Rumpel-Leed’s test, otherwise known as a tourniquet test. This test involves inflating a blood pressure cuff on your forearm for 5-10 minutes and then looking for small red or purple spots called petechiae. Over fifteen petechiae in a single area could suggest that your capillaries, small blood vessels, are more fragile than normal.

Another helpful tool is called dermoscopy. This involves using a magnifying lens to examine your skin in detail. In this condition, it can often reveal various patterns and colors on the skin.

Although the disease itself doesn’t really cause any changes in routine lab tests, your doctor might still suggest a blood test to rule out other conditions that could affect your gameplay or thrombocytes (tiny blood cells that help stop bleeding by clotting blood), and tests evaluating your blood’s ability to clot. They might also test for certain antibodies, which are proteins your body produces to fight off viruses and bacteria.

In some cases, a skin biopsy might be done for further examination. This would show certain patterns under the microscope such as infiltration of T-cells (a type of immune cell) around small blood vessels and deposition of a substance called hemosiderin, a byproduct of broken-down blood, in certain cells called macrophages in the upper layer of the skin.

Treatment Options for Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

Schamberg disease is a harmless and chronic condition which is often misunderstood due to its symptoms. Treatment for this ailment can sometimes be ineffective, and the condition may recur. It doesn’t put a person’s life at risk, nor does it pose any severe health problems. The physical symptoms of the disease can sometimes take years to fade.

First and foremost, it’s important not to worry. Dealing with Schamberg disease involves making simple lifestyle changes and incorporating a few regular treatments. Removing certain factors that may trigger the disease, like changing medication, staying away from food preservatives and artificial coloring, or even wearing support stockings can be helpful.

To help with the itching, moisturizing the skin, taking oral antihistamines, and applying topical steroids, like cortisone or betamethasone, are often recommended. Topical steroids work to reduce inflammation by controlling certain types of white blood cells that cause inflammation and helping to keep the small blood vessels in your skin stable. The idea is to get your immune system to stop attacking your skin, which is why anti-inflammatory drugs are often used in treating skin conditions like Schamberg disease.

If the first line of treatment doesn’t seem to work, doctors may recommend Pentoxifylline, a type of drug that has numerous actions. It has strong properties that improve blood flow and fight inflammation, can balance the immune system, and can reduce how much fluid leaks out of blood vessels into the skin. It does this by using different pathways, all aimed at reducing inflammation in your skin. Clinical trials showed that improvement can start anywhere from the second to third week of using Pentoxifylline, and can keep getting better over six months with regular usage.

Cases that don’t respond to these treatments can be helped with phototherapy. Phototherapy uses ultraviolet light to manipulate how the immune system works in your skin. It was seen to be useful in a few resistant cases where patients were given the therapy a couple of times a week up to 20 treatments. The treatment was successful, and for these patients, there were no recurrences during the follow-up period.

Other treatments have been used and have shown success, such as antioxidant-rich substances like ascorbic acid and bioflavonoids, which help protect blood vessels and reduce inflammation. Similar success was seen with Colchicine, Aminaphtone, and Griseofulvin – all drugs that help with blood flow, reducing inflammation, or boosting your immune system. Other drugs like cyclosporin, calcineurin-inhibitors, and methotrexate may be used, but results can vary.

Remember, Schamberg disease is a chronic but harmless condition. Treatment usually takes time, and a combination of lifestyle changes and medication therapy will be necessary to manage this ailactly. However, with patience and persistence, you can see an improvement in the condition.

What else can Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura) be?

Differentiating other skin conditions from this particular one is important, and these should notably be those that show similar symptoms like tiny, round spots that appear on the skin (petechiae), purple or red discolorations (purpura), or dark patches related to iron deposits, also known as hemosiderin. These conditions include:

• Purpura annularis telangiectodes (Majocci disease), which features circular, purple spots, slight skin thinning, and red pinpoint spots, with a clear center.
• Touraine, a variant of Majocci’s Disease, which generally has fewer, larger, and irregular rounded purple spots.
• Pigmented purpuric dermatosis of Gougerot and Blum, which has red-brown skin bumps that can merge into larger patches, especially in older men.
• Eczematoid like purpura of Doucas and Kapetanakis, which usually affects both sides of the body, is extremely itchy, and has skin changes similar to eczema. It may also show mild skin thickening and can sometimes improve on its own.
• Stasis dermatitis, which has skin patches that eventually turn to reddish-brown or brownish irregular patches. It can also include varicose veins and swelling in the legs. It can exist together with this condition.
• Leukocytoclastic vasculitis, which can be differentiated by blood-filled blisters and brown pigmentation, the tangible nature of the lesions, and certain findings in tissue examinations.
• Nonaccidental injury, which presents a purple patch that develops and resolves within a few weeks.
• Mycosis fungoides, which can be difficult to single out, but large groups of lymphocytes, or many white blood cells in the skin layer can point to mycosis fungoides.
• Scurvy, where lesions can appear similar, other signs in scurvy such as purple spots around hair follicles, twisted hairs, bleeding gums, and a history of insufficient vitamin C intake can guide towards a diagnosis of scurvy.
• Drug reactions due to medicines like rituximab, carbamazepine, meprobamate, bufexamac, chlordiazepoxide, furosemide, nitroglycerin, vitamin B-1, or medroxyprogesterone acetate.

What to expect with Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

The condition usually lasts for a long time, with many periods of worsening and improving symptoms. Sores may persist or spread over time but they can also heal on their own in a span of months to years. Based on a report by Ratnam et al, in a series of patients, 62% cleared up entirely, while 38% either remained the same or had their symptoms get worse.

Possible Complications When Diagnosed with Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

Seborrheic dermatitis (SD) is generally a harmless skin condition. However, it can cause recurrent and worsening skin issues that don’t respond well to treatment.

There are rare cases of patients with SD developing a type of skin cancer called mycosis fungoides. These conditions can look similar, both visually and under a microscope. It’s quite uncommon for mycosis fungoides to develop after an SD diagnosis, but it might be suspected if the SD symptoms persist for more than a year. Regular check-ups every year may be necessary.

Having long-lasting skin pigmentation changes due to SD can cause emotional stress and psychological trauma. Some patients may develop depression and anxiety as a result.

Key Points:

• Seborrheic dermatitis is generally harmless but can cause recurring skin problems.
• It’s rare, but SD can develop into a type of skin cancer called mycosis fungoides.
• Patients with long-lasting SD may need annual check-ups.
• Some patients may develop emotional and psychological issues due to skin pigmentation changes.

Preventing Schamberg Disease (progressive pigmentary dermatosis of Schamberg, purpura pigmentosa progressive and, Schamberg’s purpura)

It’s recommended to keep the leg elevated. This can help with swelling and pain.

A compression bandage can be used to help manage venous stasis, a condition where there is insufficient blood return from the veins in the legs to the heart. These bandages apply pressure to your leg to help blood flow.

Patients should avoid long periods of sitting or standing, also known as prolonged leg dependency, because this can make the condition worse.

Additionally, patients should avoid eating foods containing preservatives and artificial colors. These ingredients have been known to trigger the condition.