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Anetoderma

Updated : September 5, 2023





Background

Anetoderma is a rare skin disorder characterized by the presence of localized areas of flaccid skin that lack elasticity and feel soft and doughy to the touch. The term “anetoderma” comes from the Greek words “anetos,” meaning slack, and “derma,” meaning skin. The condition was first described in 1892 by French dermatologist Louis-Anne-Jean Brocq.

Anetoderma can occur in both sexes and at any age, but it is more commonly seen in women in their second to fourth decades of life. The exact cause of anetoderma is not known, although it has been associated with a variety of factors, including autoimmune diseases, viral infections, and exposure to certain medications and chemicals.

There are two main types of anetoderma: primary and secondary. Primary anetoderma occurs spontaneously without any underlying cause, while secondary anetoderma is associated with a known underlying condition or trigger.

Epidemiology

Anetoderma is a rare skin disorder, and its exact prevalence is not known. It can occur in both sexes and at any age, although it is more commonly seen in women in their second to fourth decades of life. Anetoderma has been reported in individuals of all races and ethnicities, and there is no known geographic predilection.

The condition can occur sporadically or in familial clusters, suggesting that there may be a genetic component to its development in some cases. However, most cases of anetoderma are thought to occur sporadically. Overall, anetoderma is a relatively uncommon skin condition, and more research is needed to fully understand its epidemiology and risk factors.

Anatomy

Pathophysiology

The pathophysiology of anetoderma is not fully understood, but it is thought to involve a disruption of the elastic fibers in the dermis, which are responsible for the skin’s elasticity and ability to stretch and recoil. This disruption can occur as a result of a variety of factors, including inflammation, autoimmune processes, viral infections, and exposure to certain medications and chemicals.

In primary anetoderma, the exact cause of the elastic fiber disruption is unknown, although it may be related to an autoimmune process or genetic factors. In secondary anetoderma, elastic fiber disruption is thought to be a consequence of an underlying condition or trigger, such as a viral infection, autoimmune disease, or exposure to certain medications or chemicals.

Histologically, anetoderma is characterized by a loss of elastic fibers in the affected areas, as well as the presence of inflammatory cells and altered collagen fibers. These changes can result in the characteristic soft, doughy, and wrinkled appearance of the affected skin. Overall, the pathophysiology of anetoderma is complex and needs to be fully understood. More research is needed to better understand the underlying mechanisms involved in the development of this rare skin disorder.

Etiology

The exact etiology of anetoderma is not fully understood, but it is thought to involve a combination of genetic, autoimmune, infectious, and environmental factors. In primary anetoderma, the cause is unknown, but genetic factors may play a role in some cases. Mutations in genes involved in the formation and maintenance of elastic fibers in the skin have been identified in some individuals with anetoderma, suggesting that there may be a genetic predisposition to the condition in some cases.

In secondary anetoderma, the condition is associated with underlying conditions or triggers that can cause damage to the elastic fibers in the skin. These underlying conditions may include autoimmune diseases such as lupus or scleroderma, viral infections such as hepatitis C, exposure to certain medications or chemicals, and trauma or injury to the skin. Overall, the etiology of anetoderma is complex and multifactorial, and more research is needed to fully understand the underlying causes and risk factors associated with this rare skin disorder.

Genetics

Prognostic Factors

The prognosis of anetoderma varies depending on the underlying cause and the severity of the condition. In primary anetoderma, where there is no underlying cause, the condition is usually benign and does not affect a person’s overall health. However, it can be cosmetically concerning and may cause emotional distress. In some cases, new lesions may continue to develop over time, which can further impact a person’s quality of life.

In secondary anetoderma, where an underlying condition or trigger is identified, the prognosis depends on the management of the underlying condition. With appropriate treatment, the underlying condition may be controlled, and the anetoderma lesions may improve or resolve. Overall, anetoderma is a rare and chronic condition, and there is no known cure.

However, with appropriate management strategies, it is possible to control symptoms and improve the appearance of the affected skin. Close follow-up with a dermatologist or other healthcare provider with experience in treating rare skin conditions is essential for managing anetoderma effectively.

Clinical History

Clinical History

The clinical history of anetoderma may vary depending on the type and underlying cause of the condition. In primary anetoderma, which is the more common type, there is no underlying condition or trigger, and the skin changes may appear spontaneously or gradually over time. The lesions of primary anetoderma typically appear as small, soft, raised areas of skin that eventually flatten and become wrinkled, giving the affected skin a soft, doughy, or “baggy” appearance.

The lesions are usually painless and do not cause any other symptoms. In secondary anetoderma, the skin changes may be preceded by an underlying condition or trigger, such as a viral infection or exposure to certain medications or chemicals. The lesions of secondary anetoderma may also appear spontaneously, and they typically present as small, soft, flat areas of skin with a wrinkled or puckered appearance. In some cases, the lesions may be itchy or tender. T

he onset of anetoderma is typically gradual, and the lesions may appear and disappear spontaneously or persist for long periods of time. The condition may affect any area of the body, but it most commonly affects the trunk, arms, and legs. Overall, an accurate diagnosis of anetoderma requires a thorough evaluation by a dermatologist or other healthcare provider with experience in diagnosing and treating rare skin conditions. The clinical history, along with the physical examination and diagnostic tests, can help to differentiate anetoderma from other skin conditions with similar clinical features.

Physical Examination

Physical examination

During a physical examination for anetoderma, the healthcare provider will typically evaluate the skin lesions and surrounding skin for characteristics such as size, shape, color, texture, and distribution. The lesions of anetoderma are typically small, soft, flat areas of skin with a wrinkled or puckered appearance, which may range in size from a few millimeters to several centimeters.

The lesions may be single or multiple and may occur in clusters or scattered throughout the affected area. The healthcare provider may also perform a skin biopsy, which involves removing a small sample of skin tissue for laboratory analysis. The biopsy can help to confirm the diagnosis of anetoderma by identifying the characteristic loss of elastic fibers in the skin.

If an underlying condition or trigger is suspected, the healthcare provider may also perform additional diagnostic tests such as blood tests, imaging studies, or other specialized tests to evaluate for the presence of underlying diseases or disorders. Overall, a thorough physical examination and diagnostic evaluation by a healthcare provider with experience in diagnosing and treating rare skin conditions are essential for an accurate diagnosis of anetoderma.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential diagnosis

Anetoderma can be difficult to diagnose because it shares clinical features with other skin conditions. The differential diagnosis for anetoderma includes:

  • Atrophoderma of Pasini and Pierini: This condition is characterized by flat, depressed areas of skin that may be darker in color than the surrounding skin. It is also associated with the loss of elastic fibers in the skin, but it typically affects larger areas of skin than anetoderma.
  • Lichen sclerosis: This is a chronic inflammatory skin condition that can cause skin thinning and loss of elasticity, similar to anetoderma. However, lichen sclerosis usually affects the genital area, and it is more common in women.
  • Cutaneous T-cell lymphoma: This is a type of skin cancer that can cause skin changes that may be mistaken for anetoderma. It is more common in older adults and may be associated with other symptoms such as itching or rash.
  • Connective tissue disorders: Some connective tissue disorders, such as systemic sclerosis or lupus, can cause skin changes similar to anetoderma. However, these conditions are usually associated with other systemic symptoms such as joint pain or fatigue.
  • Localized scleroderma: This is a rare autoimmune disorder that can cause skin thickening and tightening, as well as loss of elasticity, in localized areas of the body.

Overall, an accurate diagnosis of anetoderma requires a thorough evaluation by a dermatologist or other healthcare provider with experience in diagnosing and treating rare skin conditions.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

There is no cure for anetoderma, but management strategies can help to control symptoms and improve the appearance of the affected skin. The management of anetoderma depends on the underlying cause if one is identified. In cases of primary anetoderma, where there is no underlying cause, treatment is typically focused on managing symptoms, such as improving the skin’s appearance and preventing further lesions from developing.

The following are some management strategies that may be recommended for anetoderma:

  • Topical treatments: The use of topical treatments such as retinoids, topical corticosteroids, or tacrolimus ointment may help to improve the appearance of the affected skin and reduce inflammation.
  • Phototherapy: Exposure to ultraviolet light, either through natural sunlight or artificial sources, may help to improve the appearance of the affected skin and reduce the number and severity of lesions.
  • Excision and skin grafting: In severe cases, excision and skin grafting may be considered to remove the affected skin and replace it with healthy skin from another area of the body.
  • Management of underlying conditions: In cases of secondary anetoderma, where an underlying condition or trigger is identified, management strategies will focus on treating the underlying condition or removing the trigger.
  • Lifestyle modifications: Making lifestyle modifications such as avoiding sun exposure, using sunscreen, and wearing protective clothing can help to prevent further skin damage and the development of new lesions.

Overall, the management of anetoderma requires a personalized approach that takes into account the individual’s medical history, symptoms, and preferences. A dermatologist or other healthcare provider with experience in treating rare skin conditions can provide guidance and support in developing an appropriate management plan.

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/NBK560605/

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Anetoderma

Updated : September 5, 2023




Anetoderma is a rare skin disorder characterized by the presence of localized areas of flaccid skin that lack elasticity and feel soft and doughy to the touch. The term “anetoderma” comes from the Greek words “anetos,” meaning slack, and “derma,” meaning skin. The condition was first described in 1892 by French dermatologist Louis-Anne-Jean Brocq.

Anetoderma can occur in both sexes and at any age, but it is more commonly seen in women in their second to fourth decades of life. The exact cause of anetoderma is not known, although it has been associated with a variety of factors, including autoimmune diseases, viral infections, and exposure to certain medications and chemicals.

There are two main types of anetoderma: primary and secondary. Primary anetoderma occurs spontaneously without any underlying cause, while secondary anetoderma is associated with a known underlying condition or trigger.

Anetoderma is a rare skin disorder, and its exact prevalence is not known. It can occur in both sexes and at any age, although it is more commonly seen in women in their second to fourth decades of life. Anetoderma has been reported in individuals of all races and ethnicities, and there is no known geographic predilection.

The condition can occur sporadically or in familial clusters, suggesting that there may be a genetic component to its development in some cases. However, most cases of anetoderma are thought to occur sporadically. Overall, anetoderma is a relatively uncommon skin condition, and more research is needed to fully understand its epidemiology and risk factors.

The pathophysiology of anetoderma is not fully understood, but it is thought to involve a disruption of the elastic fibers in the dermis, which are responsible for the skin’s elasticity and ability to stretch and recoil. This disruption can occur as a result of a variety of factors, including inflammation, autoimmune processes, viral infections, and exposure to certain medications and chemicals.

In primary anetoderma, the exact cause of the elastic fiber disruption is unknown, although it may be related to an autoimmune process or genetic factors. In secondary anetoderma, elastic fiber disruption is thought to be a consequence of an underlying condition or trigger, such as a viral infection, autoimmune disease, or exposure to certain medications or chemicals.

Histologically, anetoderma is characterized by a loss of elastic fibers in the affected areas, as well as the presence of inflammatory cells and altered collagen fibers. These changes can result in the characteristic soft, doughy, and wrinkled appearance of the affected skin. Overall, the pathophysiology of anetoderma is complex and needs to be fully understood. More research is needed to better understand the underlying mechanisms involved in the development of this rare skin disorder.

The exact etiology of anetoderma is not fully understood, but it is thought to involve a combination of genetic, autoimmune, infectious, and environmental factors. In primary anetoderma, the cause is unknown, but genetic factors may play a role in some cases. Mutations in genes involved in the formation and maintenance of elastic fibers in the skin have been identified in some individuals with anetoderma, suggesting that there may be a genetic predisposition to the condition in some cases.

In secondary anetoderma, the condition is associated with underlying conditions or triggers that can cause damage to the elastic fibers in the skin. These underlying conditions may include autoimmune diseases such as lupus or scleroderma, viral infections such as hepatitis C, exposure to certain medications or chemicals, and trauma or injury to the skin. Overall, the etiology of anetoderma is complex and multifactorial, and more research is needed to fully understand the underlying causes and risk factors associated with this rare skin disorder.

The prognosis of anetoderma varies depending on the underlying cause and the severity of the condition. In primary anetoderma, where there is no underlying cause, the condition is usually benign and does not affect a person’s overall health. However, it can be cosmetically concerning and may cause emotional distress. In some cases, new lesions may continue to develop over time, which can further impact a person’s quality of life.

In secondary anetoderma, where an underlying condition or trigger is identified, the prognosis depends on the management of the underlying condition. With appropriate treatment, the underlying condition may be controlled, and the anetoderma lesions may improve or resolve. Overall, anetoderma is a rare and chronic condition, and there is no known cure.

However, with appropriate management strategies, it is possible to control symptoms and improve the appearance of the affected skin. Close follow-up with a dermatologist or other healthcare provider with experience in treating rare skin conditions is essential for managing anetoderma effectively.

Clinical History

The clinical history of anetoderma may vary depending on the type and underlying cause of the condition. In primary anetoderma, which is the more common type, there is no underlying condition or trigger, and the skin changes may appear spontaneously or gradually over time. The lesions of primary anetoderma typically appear as small, soft, raised areas of skin that eventually flatten and become wrinkled, giving the affected skin a soft, doughy, or “baggy” appearance.

The lesions are usually painless and do not cause any other symptoms. In secondary anetoderma, the skin changes may be preceded by an underlying condition or trigger, such as a viral infection or exposure to certain medications or chemicals. The lesions of secondary anetoderma may also appear spontaneously, and they typically present as small, soft, flat areas of skin with a wrinkled or puckered appearance. In some cases, the lesions may be itchy or tender. T

he onset of anetoderma is typically gradual, and the lesions may appear and disappear spontaneously or persist for long periods of time. The condition may affect any area of the body, but it most commonly affects the trunk, arms, and legs. Overall, an accurate diagnosis of anetoderma requires a thorough evaluation by a dermatologist or other healthcare provider with experience in diagnosing and treating rare skin conditions. The clinical history, along with the physical examination and diagnostic tests, can help to differentiate anetoderma from other skin conditions with similar clinical features.

Physical examination

During a physical examination for anetoderma, the healthcare provider will typically evaluate the skin lesions and surrounding skin for characteristics such as size, shape, color, texture, and distribution. The lesions of anetoderma are typically small, soft, flat areas of skin with a wrinkled or puckered appearance, which may range in size from a few millimeters to several centimeters.

The lesions may be single or multiple and may occur in clusters or scattered throughout the affected area. The healthcare provider may also perform a skin biopsy, which involves removing a small sample of skin tissue for laboratory analysis. The biopsy can help to confirm the diagnosis of anetoderma by identifying the characteristic loss of elastic fibers in the skin.

If an underlying condition or trigger is suspected, the healthcare provider may also perform additional diagnostic tests such as blood tests, imaging studies, or other specialized tests to evaluate for the presence of underlying diseases or disorders. Overall, a thorough physical examination and diagnostic evaluation by a healthcare provider with experience in diagnosing and treating rare skin conditions are essential for an accurate diagnosis of anetoderma.

Differential diagnosis

Anetoderma can be difficult to diagnose because it shares clinical features with other skin conditions. The differential diagnosis for anetoderma includes:

  • Atrophoderma of Pasini and Pierini: This condition is characterized by flat, depressed areas of skin that may be darker in color than the surrounding skin. It is also associated with the loss of elastic fibers in the skin, but it typically affects larger areas of skin than anetoderma.
  • Lichen sclerosis: This is a chronic inflammatory skin condition that can cause skin thinning and loss of elasticity, similar to anetoderma. However, lichen sclerosis usually affects the genital area, and it is more common in women.
  • Cutaneous T-cell lymphoma: This is a type of skin cancer that can cause skin changes that may be mistaken for anetoderma. It is more common in older adults and may be associated with other symptoms such as itching or rash.
  • Connective tissue disorders: Some connective tissue disorders, such as systemic sclerosis or lupus, can cause skin changes similar to anetoderma. However, these conditions are usually associated with other systemic symptoms such as joint pain or fatigue.
  • Localized scleroderma: This is a rare autoimmune disorder that can cause skin thickening and tightening, as well as loss of elasticity, in localized areas of the body.

Overall, an accurate diagnosis of anetoderma requires a thorough evaluation by a dermatologist or other healthcare provider with experience in diagnosing and treating rare skin conditions.

There is no cure for anetoderma, but management strategies can help to control symptoms and improve the appearance of the affected skin. The management of anetoderma depends on the underlying cause if one is identified. In cases of primary anetoderma, where there is no underlying cause, treatment is typically focused on managing symptoms, such as improving the skin’s appearance and preventing further lesions from developing.

The following are some management strategies that may be recommended for anetoderma:

  • Topical treatments: The use of topical treatments such as retinoids, topical corticosteroids, or tacrolimus ointment may help to improve the appearance of the affected skin and reduce inflammation.
  • Phototherapy: Exposure to ultraviolet light, either through natural sunlight or artificial sources, may help to improve the appearance of the affected skin and reduce the number and severity of lesions.
  • Excision and skin grafting: In severe cases, excision and skin grafting may be considered to remove the affected skin and replace it with healthy skin from another area of the body.
  • Management of underlying conditions: In cases of secondary anetoderma, where an underlying condition or trigger is identified, management strategies will focus on treating the underlying condition or removing the trigger.
  • Lifestyle modifications: Making lifestyle modifications such as avoiding sun exposure, using sunscreen, and wearing protective clothing can help to prevent further skin damage and the development of new lesions.

Overall, the management of anetoderma requires a personalized approach that takes into account the individual’s medical history, symptoms, and preferences. A dermatologist or other healthcare provider with experience in treating rare skin conditions can provide guidance and support in developing an appropriate management plan.

https://www.ncbi.nlm.nih.gov/books/NBK560605/

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