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Schamberg's Disease- Causes, Symptoms and Treatment

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Schamberg's Disease- Causes, Symptoms and Treatment

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Schamberg's is a skin disease characterized by reddish-brown pigmentations or patches, mostly in the lower extremities. Read the below article to know more.

Medically reviewed by

Dr. Nidhin Varghese

Published At July 13, 2022
Reviewed AtJanuary 24, 2023

Introduction:

Pigmented patches in the skin are common. What makes them distinguishable is their color and appearance! One among the various patches that appear on the skin is reddish-brown patches responsible for a disease called Schamberg's. It comes under a group of conditions called progressive pigmented purpuric dermatoses. It is a form of capillaritis that mainly affects the lower limbs.

What Is Capillaritis?

  • Capillaritis is caused due to the leakage of blood from the capillaries (the tiny blood vessels that are present superficially in the skin).

  • When the capillaries get inflamed, the red blood cells tend to break, leading to the deposition of hemosiderin (a protein compound that stores iron in the tissues) which causes pigmentations in the skin.

What Are the Causes of Schamberg's Disease?

Though the apparent cause for this disease remains unknown, it could be due to any one of the following reasons;

  • Specific allergic reactions to various allergens like food or certain substances might cause this disease.

  • The pigmentations associated with this disease might be a reaction to any medications taken for the underlying conditions.

  • Schamberg's disease could be due to any active viral infections.

  • Hereditary reasons, this disease shows a genetic predisposition, which means that it runs in families.

  • Alcohol intake could also be a causative factor for this disease.

  • Exercising can be the cause too.

  • Low immune responses can trigger such lesions.

  • There is no such evidence that shows a correlation between Schamberg’s disease and hematological reasons. Although, the features are similar in both.

  • Certain underlying diseases can aggravate this disease, like diabetes, hypertension, capillary, fragility, etc.

Who Is Prone to Schamberg's Disease?

  • The otherwise healthy people are usually affected.

  • It is stated that males are more susceptible to this disease, though it affects females.

  • Schamberg's disease is more common in middle-aged to older people than in children.

What Are the Characteristic Features of Schamberg's Disease?

  • It is an entirely benign condition which means it is not life-threatening.

  • It is chronic (present for a more extended period) and usually is present for a lifetime with an intermittent flare-up. The spots keep fading away with time, and the intensity of the color varies based on their time of onset.

  • Usually appears as regular or irregular reddish-brown patches with Cayenne pepper-like spots along its borders, which is a prominent feature of this condition. They form papules or macules-like lesions; it keeps progressing with time.

  • Predominantly they are localized to the lower extremities and spread to the thighs and buttocks, but can also appear in the other body parts such as the upper extremities and trunk, or they can be generalized, which is relatively uncommon. It usually does not affect the face.

  • It affects the body parts bilaterally.

  • They may present as a single patch or can be multiple.

  • These are not palpable, and the size typically of each macule is not more than 3 centimetres (cm).

  • These patches tend to recur.

Why Are the Spots Reddish-Brown in Color?

  • Hemosiderin staining is the reason for this condition's reddish, orangish, brownish pigmentations.

  • In the case of inflamed capillaries, blood seeps out, causing breakage of the red blood cells into the skin and releasing iron from the hemoglobin present in the red blood cells that get deposited as hemosiderin under the skin, causing pigmentations that are reddish-brown.

  • The white blood cells, called the immune cells, can usually remove the deposited hemosiderin to an extent, but in conditions like Schamberg's, they fail to remove it.

  • In some cases, the intensity of the pigment varies with time. The pigmentations that have been present for years show pale pigments due to the reabsorption of hemosiderin.

What Are the Symptoms of Schamberg’s Disease?

Usually, this condition is entirely asymptomatic; very rarely can show pruritus (itchy skin) or pain.

How Is Schamberg's Disease Diagnosed?

  • This disease is diagnosed by closely examining the lesion clinically and correlating the features present.

  • Dermoscopy can be used as a diagnostic tool as it uses a unique lens to view the lesion.

  • This disease does not show any abnormalities in the blood test; blood tests like complete blood count peripheral smear are done to rule out other conditions associated with blood presenting similar features.

  • A biopsy is not necessary to diagnose this disease but is performed to differentiate it from other similar conditions and confirm the diagnosis.

How Is Schamberg's Disease Treated?

As the reason for this disease still stands a query, there is no specific treatment for this condition. Various treatments have been tried, and few have shown significant results.

Few treatment options include the following;

  • Discontinuing the drugs that occurred as the trigger factor, one should avoid any allergen or food that caused the lesion.

  • It is initially treated using topical steroid ointments.

  • Wearing compression stockings to compensate for the abnormalities in the capillaries.

  • Phototherapy is seen to provide promising results.

  • The antioxidant properties of Rutoside and Ascorbic acid have shown proven results.

  • Narrowband UV light has also been used as a treatment option.

  • In superimposed infection (lesions that get infected), antibiotics are given to minimize the infection.

  • Also, advanced fluorescent technology is a mode of treatment for this disease.

  • Few lasers used for aesthetic and cosmetological purposes have also shown results.

How Is the Prognosis of Schamberg's Disease After Treatment?

This disease usually shows spontaneous healing but has remained persistent without recovery and subsequent worsening in a few cases. Due to its chronic nature, it stays for an extended period. It has also shown recurrence in some cases even after the treatment.

Conclusion:

This disease does not necessarily have to provoke your fear factor as it is danger-free and has shown spontaneous healing in most cases. Most people get treated for this condition owing to its displeasing appearance aesthetically. Though the disease does not have a specific cause or a proper treatment protocol, it has responded to various treatment modalities, excluding a few.

Frequently Asked Questions

1.

How to Treat Schamberg's Disease?

The reddish-brown patches caused by Schamberg’s disease are treated as follows:
- Topical steroids.
- Avoid foods and drugs that may cause allergic reactions.
- Try wearing stockings, as it compensates for capillary abnormalities.
- Oral antihistamines.
- Phototherapy.
- Ascorbic acid.
- Other drugs like Colchicine, Aminaphtone, and Griseofulvin are also used in treating Schamberg’s disease.

2.

Can Medications Cause Schamberg’s Disease?

Schamberg’s disease may occur due to various reasons. Of which the following drugs are also involved in causing the peculiar reddish-brown patches:
- Amlodipine.
- Glipizide.
- Hydralazine.
- Nitroglycerin.
- Thiamine.
- Topical Fluorouracil.
- Acetaminophen.
- Aspirin, etc.

3.

What Are the Features of Schamberg's Disease?

The characteristic signs and symptoms of Schamberg’s disease are as follows:
- Mostly asymptomatic.
- Itchy or painful lesions may occur on the lower legs.
- Irregular reddish-brown patches that are well-circumscribed may develop.
- The common sites include - Thighs, lower legs, buttocks, and, less commonly, the upper extremities.
- Chronic in nature, and the lesions may recur.
- Flare-ups are common.

4.

Does Alcohol Cause Schamberg’s Disease?

Several studies report that alcohol intake is closely associated with Schamberg’s disease. Due to the fact that the development of hypersensitivity reaction after heavy alcohol use. In addition, the consumption of alcohol for a long time results in alcoholic liver disease; Schamberg’s disease also occurs as a late complication. However, these lesions are benign and are not life-threatening.

5.

How Common Is Schamberg’s Disease?

Schamberg’s disease is a chronic condition and a type of pigmented purpuric dermatoses (PPD). It causes yellow or reddish-brown patches in the lower extremities, buttocks, and thighs. In addition, the disease is uncommon, and reports state that men are more commonly affected than women. Schamberg’s disease may develop at any age; however, the middle-aged population is more affected.

6.

Is Schamberg’s Disease a Vasculitis?

Schamberg’s disease occurs due to blood vessel leakage that results in red blood cells (RBCs) deposition over the skin. The RBCs containing hemoglobin release the iron, causing a reddish-brown rash on the skin. Schamberg’s disease is a type of vascular disorder, not vasculitis. Vasculitis is characterized by the inflammation of the vessels, thus reducing the width of blood vessels. It results in decreased blood flow to various organs causing their damage.

7.

Does Taking Acetaminophen Cause Schamberg’s Disease?

Until now, no studies have proven the exact cause of Schamberg’s disease. However, there is research stating that certain drugs may trigger hypersensitivity reactions in the skin, causing characteristics of reddish-brown, itchy patches of Schamberg’s disease. Among them, intake of Acetaminophen has triggered the occurrence of pin-headed spots over the lower extremities. However, these skin abnormalities clear after a few weeks of Acetaminophen cessation.

8.

Is Schamberg’s Disease Associated With Thyroid Disorder?

Hypothyroidism is a thyroid disorder causing a decreased release of thyroid hormone resulting in improper growth and development. It is more common among women and causes dry skin, weight gain, cold sensitivity, fatigue, etc. In addition, a few studies report that Schamberg’s disease, a type of skin disorder, may occur in women with hypothyroidism. The exact mechanism is unknown; however, the immunological changes might trigger Schamberg’s disease.

9.

Is Schamberg’s Disease a Hereditary Disorder?

Schamberg’s disease belongs to the pigmented purpuric dermatoses (PPD) group and causes skin pigmentations. It is a chronic condition and keeps recurring. It may occur due to various causes, and several studies show that individuals with a family history of Schamberg’s disease may also develop it. But it is uncommon to occur so. In addition, the treatment depends on the factors causing it.

10.

Is Schamberg’s Disease Life-Threatening?

Schamberg’s disease is a chronic skin disorder, and it keeps recurring. However, they are not life-threatening. But, it does not mean they are entirely treatable, and they may cause skin pigmentation, itching, and sometimes pain in the lower extremities, buttocks, and thighs. In addition, it is crucial to identify the causative factor to achieve the complete effectiveness of the treatment.

11.

Is Schamberg’s Disease Considered a Bleeding Disorder?

Schamberg’s disease is not a bleeding disorder. But, the rupture of blood vessels causes an accumulation of red blood cells (RBC’s) along the skin. After that, the RBCs tend to secrete the iron from them, causing brownish pigmentation on the skin. In contrast, bleeding disorders are characterized by extended bleeding post-injury or surgery. It may occur in joints, muscles, or any soft tissues. The specialist may decide the treatment depending on the severity of the bleeding disorder.
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Dr. Nidhin Varghese
Dr. Nidhin Varghese

Dermatology

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