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Schamberg Disease

Updated : April 4, 2023





Background

Schamberg’s disease, also known as progressive pigmentary dermatosis of Schamberg, is a rare chronic skin disorder characterized by the appearance of small, flat, reddish-brown or purple lesions on the legs or feet. The condition is named after Jay Frank Schamberg, an American dermatologist who first described it in 1901.

Schamberg’s disease is believed to be caused by an abnormality in the small blood vessels (capillaries) beneath the skin, which leads to leakage of red blood cells and deposition of hemosiderin pigment. It is more common in males and typically develops in adulthood, although it can affect people of all ages. The condition is not contagious, and the lesions are usually asymptomatic, although some people may experience mild itching or burning.

Epidemiology

Schamberg’s disease is a rare condition, and its prevalence is not well known. It is more commonly reported in males than females and can affect people of all ages, although it typically develops in adulthood. The condition is more prevalent in certain populations, such as people of Asian or African descent, and may be more common in warm climates.

Some studies have suggested a genetic predisposition to the disease, although more research is needed to understand the underlying causes. Overall, Schamberg’s disease is considered a benign condition and does not significantly impact a person’s health or life expectancy. However, it can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Anatomy

Pathophysiology

The exact pathophysiology of Schamberg’s disease is not fully understood, but it is believed to involve an abnormality in the small blood vessels (capillaries) beneath the skin. This can result in the leakage of red blood cells and deposition of hemosiderin pigment, leading to the characteristic reddish-brown or purple lesions that are seen in the affected areas. The underlying cause of the capillary damage is not clear, but it is thought to be related to an immune response or inflammation.

Some researchers have suggested that the immune system may be attacking the blood vessels in the affected areas, leading to their breakdown and leakage of red blood cells. Other factors that may contribute to the development of Schamberg’s disease include chronic venous insufficiency, exposure to certain chemicals or medications, and viral infections.

It is also believed that some cases may be inherited or have a genetic component. The lesions associated with Schamberg’s disease are typically asymptomatic, although some people may experience mild itching or burning. In most cases, the condition does not progress beyond the appearance of the lesions and does not pose a significant risk to a person’s health.

Etiology

The exact etiology of Schamberg’s disease is not fully understood, but it is believed to be multifactorial and may involve a combination of genetic, immunological, and environmental factors. Some studies have suggested that the condition may be caused by an abnormal immune response that leads to inflammation and damage to the blood vessels in the skin.

Other researchers have suggested that certain medications, chemicals, or viral infections may trigger the condition. In some cases, Schamberg’s disease may be inherited or have a genetic component. A mutation in the CYP2C9 gene has been identified in some families with a history of the condition, although not all cases of Schamberg’s disease are hereditary.

Other factors that may contribute to the development of Schamberg’s disease include chronic venous insufficiency, which can cause blood to pool in the legs and damage the capillaries, and exposure to certain chemicals or metals, such as nickel or rubber, which can trigger an allergic reaction in the skin. Overall, the exact etiology of Schamberg’s disease remains unclear, and more research is needed to better understand the underlying causes of this rare skin condition.

Genetics

Prognostic Factors

The prognosis for Schamberg’s disease is generally good, as the condition is benign and self-limited. In many cases, the lesions may spontaneously resolve without treatment. However, some people may experience a chronic, relapsing course of the disease with recurrent episodes of new lesions. While Schamberg’s disease does not pose a significant risk to a person’s health, the appearance of the lesions can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Treatment can be effective in reducing the appearance of the lesions and managing associated symptoms such as itching and dryness. It is important for individuals with Schamberg’s disease to avoid factors that can exacerbate the condition, such as tight clothing, exposure to irritants, and prolonged standing or sitting.

Regular follow-up with a healthcare provider may also be recommended to monitor the progression of the disease and adjust treatment as necessary. Overall, the prognosis for Schamberg’s disease is good, and the condition typically does not have any significant long-term health consequences.

Clinical History

Clinical history

Schamberg’s disease typically presents as asymptomatic, with reddish-brown or purple lesions on the lower legs. The lesions are flat, and the affected area may have a slightly raised, rough texture. The lesions can range in size from a few millimeters to several centimeters in diameter and may appear as a single lesion or multiple lesions. The condition may progress slowly over time, with new lesions appearing and existing lesions becoming darker in color.

In some cases, the lesions may spread to involve the thighs or other areas of the body, although this is rare. Although the lesions associated with Schamberg’s disease are usually asymptomatic, some people may experience mild itching or burn in the affected areas. The lesions are typically not painful and do not cause any other systemic symptoms. The onset of Schamberg’s disease is typically gradual, and the lesions may persist for months to years without treatment.

In some cases, the lesions may resolve spontaneously, although this is uncommon. A thorough clinical history and physical examination are usually sufficient to diagnose Schamberg’s disease, although a biopsy may be necessary in some cases to rule out other skin conditions. The condition is typically benign and does not pose a significant risk to a person’s health, although it can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Physical Examination

Physical examination

During physical examination, Schamberg’s disease typically presents as reddish-brown or purple lesions on the lower legs, although the lesions may also involve the thighs or other areas of the body. The lesions are usually flat and well-demarcated, with a slightly raised, rough texture. They may be small or large and can appear as single lesions or multiple lesions. The lesions may be distributed symmetrically or asymmetrically and may have a reticulated or cayenne pepper-like appearance.

In some cases, the lesions may have a more linear or streaky pattern. The affected area may be hyperpigmented, with a reddish-brown or purplish hue, and there may be evidence of hemosiderin deposition in the skin. Hemosiderin is a pigment that results from the breakdown of red blood cells and can cause a brownish discoloration in the skin. In most cases, the lesions associated with Schamberg’s disease are asymptomatic, although some people may experience mild itching or burn in the affected areas.

The diagnosis of Schamberg’s disease is usually based on the characteristic appearance of the lesions and a thorough clinical history, although a skin biopsy may be necessary in some cases to rule out other skin conditions. Overall, Schamberg’s disease is a benign condition that does not pose a significant risk to a person’s health, although it can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential diagnosis

Schamberg’s disease can resemble other skin conditions, so it is important to consider a differential diagnosis when evaluating a patient with skin lesions.

Some conditions that may be considered in the differential diagnosis of Schamberg’s disease include:

  • Contact dermatitis: This is an allergic reaction to an irritant or allergen that causes redness, itching, and swelling in the affected area.
  • Pigmented purpuric dermatoses: This group of skin conditions includes Schamberg’s disease, as well as other conditions that cause reddish-brown or purple lesions on the skin. These conditions are believed to be related and may share a similar pathophysiology.
  • Lichen planus: This is an inflammatory condition that can cause red, itchy bumps or lesions on the skin.
  • Erythema multiforme: This is an inflammatory skin condition that can cause reddish-brown or purple lesions on the skin, often in a target-like pattern.
  • Vasculitis: This is an inflammatory condition that affects the blood vessels and can cause redness, swelling, and damage to the skin and other organs.
  • Purpura: This is a condition that causes bleeding under the skin, resulting in purple or reddish-brown discoloration.
  • Spider angioma: This is a type of benign blood vessel tumor that can cause red or purple lesions on the skin, often with a central red spot surrounded by a spider-like network of blood vessels.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

There is no specific cure for Schamberg’s disease, but the condition is usually self-limited and benign. Treatment is primarily focused on managing symptoms and preventing the development of new lesions.

Some treatment options that may be considered include:

  • Topical corticosteroids: These medications can help reduce inflammation and itching in the affected area. They may be applied directly to the skin in the form of a cream or ointment.
  • Compression stockings: Wearing compression stockings or bandages can help improve blood flow and reduce the appearance of the lesions.
  • Moisturizers: Applying moisturizers to the affected area can help relieve dryness and itching.
  • Phototherapy: This treatment involves exposing the skin to ultraviolet light and may be effective in reducing the appearance of the lesions.
  • Laser therapy: Certain types of lasers may be used to target and destroy the blood vessels that are causing the discoloration in the affected area.
  • Oral antihistamines: These medications may be used to help relieve itching associated with the lesions.

It is important to note that while some treatments may be effective in reducing the appearance of the lesions, they may not prevent the development of new lesions or completely cure the condition. In addition to medical treatment, it is also important for individuals with Schamberg’s disease to avoid factors that can exacerbate the condition, such as tight clothing, exposure to irritants, and prolonged standing or sitting.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK560532/

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Schamberg Disease

Updated : April 4, 2023




Schamberg’s disease, also known as progressive pigmentary dermatosis of Schamberg, is a rare chronic skin disorder characterized by the appearance of small, flat, reddish-brown or purple lesions on the legs or feet. The condition is named after Jay Frank Schamberg, an American dermatologist who first described it in 1901.

Schamberg’s disease is believed to be caused by an abnormality in the small blood vessels (capillaries) beneath the skin, which leads to leakage of red blood cells and deposition of hemosiderin pigment. It is more common in males and typically develops in adulthood, although it can affect people of all ages. The condition is not contagious, and the lesions are usually asymptomatic, although some people may experience mild itching or burning.

Schamberg’s disease is a rare condition, and its prevalence is not well known. It is more commonly reported in males than females and can affect people of all ages, although it typically develops in adulthood. The condition is more prevalent in certain populations, such as people of Asian or African descent, and may be more common in warm climates.

Some studies have suggested a genetic predisposition to the disease, although more research is needed to understand the underlying causes. Overall, Schamberg’s disease is considered a benign condition and does not significantly impact a person’s health or life expectancy. However, it can cause cosmetic concerns and may be associated with psychological distress in some individuals.

The exact pathophysiology of Schamberg’s disease is not fully understood, but it is believed to involve an abnormality in the small blood vessels (capillaries) beneath the skin. This can result in the leakage of red blood cells and deposition of hemosiderin pigment, leading to the characteristic reddish-brown or purple lesions that are seen in the affected areas. The underlying cause of the capillary damage is not clear, but it is thought to be related to an immune response or inflammation.

Some researchers have suggested that the immune system may be attacking the blood vessels in the affected areas, leading to their breakdown and leakage of red blood cells. Other factors that may contribute to the development of Schamberg’s disease include chronic venous insufficiency, exposure to certain chemicals or medications, and viral infections.

It is also believed that some cases may be inherited or have a genetic component. The lesions associated with Schamberg’s disease are typically asymptomatic, although some people may experience mild itching or burning. In most cases, the condition does not progress beyond the appearance of the lesions and does not pose a significant risk to a person’s health.

The exact etiology of Schamberg’s disease is not fully understood, but it is believed to be multifactorial and may involve a combination of genetic, immunological, and environmental factors. Some studies have suggested that the condition may be caused by an abnormal immune response that leads to inflammation and damage to the blood vessels in the skin.

Other researchers have suggested that certain medications, chemicals, or viral infections may trigger the condition. In some cases, Schamberg’s disease may be inherited or have a genetic component. A mutation in the CYP2C9 gene has been identified in some families with a history of the condition, although not all cases of Schamberg’s disease are hereditary.

Other factors that may contribute to the development of Schamberg’s disease include chronic venous insufficiency, which can cause blood to pool in the legs and damage the capillaries, and exposure to certain chemicals or metals, such as nickel or rubber, which can trigger an allergic reaction in the skin. Overall, the exact etiology of Schamberg’s disease remains unclear, and more research is needed to better understand the underlying causes of this rare skin condition.

The prognosis for Schamberg’s disease is generally good, as the condition is benign and self-limited. In many cases, the lesions may spontaneously resolve without treatment. However, some people may experience a chronic, relapsing course of the disease with recurrent episodes of new lesions. While Schamberg’s disease does not pose a significant risk to a person’s health, the appearance of the lesions can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Treatment can be effective in reducing the appearance of the lesions and managing associated symptoms such as itching and dryness. It is important for individuals with Schamberg’s disease to avoid factors that can exacerbate the condition, such as tight clothing, exposure to irritants, and prolonged standing or sitting.

Regular follow-up with a healthcare provider may also be recommended to monitor the progression of the disease and adjust treatment as necessary. Overall, the prognosis for Schamberg’s disease is good, and the condition typically does not have any significant long-term health consequences.

Clinical history

Schamberg’s disease typically presents as asymptomatic, with reddish-brown or purple lesions on the lower legs. The lesions are flat, and the affected area may have a slightly raised, rough texture. The lesions can range in size from a few millimeters to several centimeters in diameter and may appear as a single lesion or multiple lesions. The condition may progress slowly over time, with new lesions appearing and existing lesions becoming darker in color.

In some cases, the lesions may spread to involve the thighs or other areas of the body, although this is rare. Although the lesions associated with Schamberg’s disease are usually asymptomatic, some people may experience mild itching or burn in the affected areas. The lesions are typically not painful and do not cause any other systemic symptoms. The onset of Schamberg’s disease is typically gradual, and the lesions may persist for months to years without treatment.

In some cases, the lesions may resolve spontaneously, although this is uncommon. A thorough clinical history and physical examination are usually sufficient to diagnose Schamberg’s disease, although a biopsy may be necessary in some cases to rule out other skin conditions. The condition is typically benign and does not pose a significant risk to a person’s health, although it can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Physical examination

During physical examination, Schamberg’s disease typically presents as reddish-brown or purple lesions on the lower legs, although the lesions may also involve the thighs or other areas of the body. The lesions are usually flat and well-demarcated, with a slightly raised, rough texture. They may be small or large and can appear as single lesions or multiple lesions. The lesions may be distributed symmetrically or asymmetrically and may have a reticulated or cayenne pepper-like appearance.

In some cases, the lesions may have a more linear or streaky pattern. The affected area may be hyperpigmented, with a reddish-brown or purplish hue, and there may be evidence of hemosiderin deposition in the skin. Hemosiderin is a pigment that results from the breakdown of red blood cells and can cause a brownish discoloration in the skin. In most cases, the lesions associated with Schamberg’s disease are asymptomatic, although some people may experience mild itching or burn in the affected areas.

The diagnosis of Schamberg’s disease is usually based on the characteristic appearance of the lesions and a thorough clinical history, although a skin biopsy may be necessary in some cases to rule out other skin conditions. Overall, Schamberg’s disease is a benign condition that does not pose a significant risk to a person’s health, although it can cause cosmetic concerns and may be associated with psychological distress in some individuals.

Differential diagnosis

Schamberg’s disease can resemble other skin conditions, so it is important to consider a differential diagnosis when evaluating a patient with skin lesions.

Some conditions that may be considered in the differential diagnosis of Schamberg’s disease include:

  • Contact dermatitis: This is an allergic reaction to an irritant or allergen that causes redness, itching, and swelling in the affected area.
  • Pigmented purpuric dermatoses: This group of skin conditions includes Schamberg’s disease, as well as other conditions that cause reddish-brown or purple lesions on the skin. These conditions are believed to be related and may share a similar pathophysiology.
  • Lichen planus: This is an inflammatory condition that can cause red, itchy bumps or lesions on the skin.
  • Erythema multiforme: This is an inflammatory skin condition that can cause reddish-brown or purple lesions on the skin, often in a target-like pattern.
  • Vasculitis: This is an inflammatory condition that affects the blood vessels and can cause redness, swelling, and damage to the skin and other organs.
  • Purpura: This is a condition that causes bleeding under the skin, resulting in purple or reddish-brown discoloration.
  • Spider angioma: This is a type of benign blood vessel tumor that can cause red or purple lesions on the skin, often with a central red spot surrounded by a spider-like network of blood vessels.

There is no specific cure for Schamberg’s disease, but the condition is usually self-limited and benign. Treatment is primarily focused on managing symptoms and preventing the development of new lesions.

Some treatment options that may be considered include:

  • Topical corticosteroids: These medications can help reduce inflammation and itching in the affected area. They may be applied directly to the skin in the form of a cream or ointment.
  • Compression stockings: Wearing compression stockings or bandages can help improve blood flow and reduce the appearance of the lesions.
  • Moisturizers: Applying moisturizers to the affected area can help relieve dryness and itching.
  • Phototherapy: This treatment involves exposing the skin to ultraviolet light and may be effective in reducing the appearance of the lesions.
  • Laser therapy: Certain types of lasers may be used to target and destroy the blood vessels that are causing the discoloration in the affected area.
  • Oral antihistamines: These medications may be used to help relieve itching associated with the lesions.

It is important to note that while some treatments may be effective in reducing the appearance of the lesions, they may not prevent the development of new lesions or completely cure the condition. In addition to medical treatment, it is also important for individuals with Schamberg’s disease to avoid factors that can exacerbate the condition, such as tight clothing, exposure to irritants, and prolonged standing or sitting.

https://www.ncbi.nlm.nih.gov/books/NBK560532/

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