Necrobiosis lipoidica (NL) is an infrequent, long-lasting, and unexplained granulomatous condition characterized by collagen degeneration. It carries an intensified risk of ulceration and is commonly linked to type 1 diabetes mellitus. The disease is characterized by thickened blood vessel walls and the accumulation of fat.
One of its primary complications is the development of ulcers, typically triggered by physical injury. Infections can also occur, although they are less common. Additionally, in rare cases, if necrobiosis lipoidica persists over time, it may transform into squamous cell carcinoma.
Epidemiology
Necrobiosis lipoidica is more commonly observed in individuals with diabetes, although the validity of this association is currently subject to debate. The incidence of necrobiosis lipoidica among people with diabetes ranges from 0.3% to 1.2%. It is important to note that necrobiosis lipoidica may occur before the onset of diabetes in approximately 14% of cases, simultaneously with diabetes in around 24% of cases and after the diagnosis of diabetes in 62% of cases.
The likelihood of developing necrobiosis lipoidica is not influenced by the degree of glycemic control, as there is no proven correlation. There is a higher prevalence of necrobiosis lipoidica in females, accounting for approximately 77% of cases, and the onset of the condition tends to occur at a younger age in women compared to men. The average age of onset for necrobiosis lipoidica is typically between 30 and 40 years.
Anatomy
Pathophysiology
One key aspect of necrobiosis lipoidica is collagen degeneration, specifically in the deep dermis and subcutaneous fat layer. The exact reason for this collagen degeneration is unclear, but it is thought to involve immune-mediated processes and abnormal inflammation. It is believed that immune dysfunction plays a role in the pathogenesis of NL. Inflammatory cells, such as T lymphocytes, macrophages, and neutrophils, infiltrate the affected skin. These immune cells release various inflammatory mediators, including cytokines and chemokines, contributing to tissue damage and inflammation.
Vascular changes are also observed in NL. The walls of small blood vessels thicken, leading to impaired blood flow and reduced oxygen supply to the affected area. This vascular abnormality may further contribute to tissue damage and the formation of characteristic lesions. The lipids accumulation in the affected skin is another characteristic feature of NL. It is believed to result from the impaired clearance of lipids due to vascular abnormalities and inflammation.
The exact role of lipid accumulation in the pathophysiology of NL is not fully understood, but it may contribute to tissue damage and inflammation. While there is an increased prevalence of NL in individuals with diabetes, the exact relationship between the two conditions remains uncertain. It is hypothesized that hyperglycemia, immune dysregulation, and vascular abnormalities associated with diabetes may play a role in the development or progression of NL in susceptible individuals.
Etiology
The etiology of necrobiosis lipoidica remains uncertain and is considered multifactorial. While the exact cause is unknown, several factors have been proposed to contribute to the development of this condition.
Immunological Factors: Immune dysfunction and abnormal immune responses are believed to play a role in the pathogenesis of NL. There is evidence of T-cell-mediated immune reactions and an increased presence of various immune cells, including T lymphocytes, macrophages, and neutrophils, within the affected skin. These immune cells release inflammatory mediators that contribute to tissue damage and inflammation.
Vascular Abnormalities: Changes in the blood vessels supplying the affected skin have been observed in NL. Thickening of the vessel walls, narrowing of the lumen, and reduced blood flow may contribute to tissue hypoxia and damage.
Collagen Degeneration: NL is characterized by the degeneration of collagen in the deep dermis and subcutaneous fat layer. The reasons for this collagen degeneration are not well-defined, but it is believed to involve alterations in collagen synthesis and degradation processes.
Genetic Predisposition: There may be a genetic susceptibility to developing NL, as some cases have been reported in families. However, specific genes or genetic mutations associated with NL have not been identified.
Association with Diabetes Mellitus: Although the relationship is not fully understood, NL is more commonly seen in individuals with diabetes, particularly type 1 diabetes. The exact mechanisms linking NL and diabetes are unclear but may involve factors such as hyperglycemia, microvascular changes, and immune dysregulation associated with diabetes.
Trauma or Injury: Trauma or injury to the skin has been reported as a triggering factor for the development of NL lesions. However, not all cases of NL are associated with preceding trauma.
Genetics
Prognostic Factors
Clinical History
Clinical History
Necrobiosis lipoidica typically presents as well-defined, round, or oval-shaped plaques on the skin. These lesions are usually located on the lower legs but can also occur in other body areas. A yellowish or reddish-brown color characterizes the plaques, which may appear shiny or atrophic. The surface of the lesions may be smooth or slightly raised. The size and distribution of the lesions can vary.
They may range from a few millimeters to several centimeters in diameter. In some cases, multiple plaques may be present, and they can coalesce to form larger areas of involvement. Patients with necrobiosis lipoidica may experience various symptoms, although some individuals may remain asymptomatic. Common symptoms include itching, burning, and pain in the affected areas.
These symptoms can vary in intensity and may be aggravated by certain triggers. The lesions’ duration and progression over time are important to assess. Necrobiosis lipoidica can be a chronic condition with periods of stability or intermittent flare-ups. Documenting the history of lesion development and changes in size, color, or symptoms can provide valuable information.
Physical Examination
Physical Examination
The primary characteristic of NL is the presence of well-defined plaques on the skin. These plaques typically have a yellowish or reddish-brown color and may appear shiny or atrophic. They can range from a few millimeters to several centimeters in diameter. The lesions are commonly located on the lower legs but can also occur in other areas of the body.
The texture of the lesions can vary. In some cases, the plaques may have a smooth surface, while in others, they may appear slightly raised or wrinkled. Over time, the lesions may become atrophic, meaning the skin in the affected area appears thin and depressed.
This atrophy can contribute to the glossy appearance of the plaques. In some cases, NL plaques may develop ulcers, especially following trauma or injury to the affected skin. These ulcers can be deep, slow to heal, and may increase the risk of infection. The skin surrounding the NL lesions may show signs of inflammation, such as erythema or edema. The patient may report itching, burning, or pain in the NL lesions.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential Diagnoses
Xanthomas
Rheumatoid Arthritis
Sarcoidosis
Granuloma Annulare
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
The necrobiosis lipoidica treatment remains challenging, as no definitive therapy has been proven effective. Glycemic control in patients with diabetes mellitus does not typically significantly impact the course of NL. A conservative approach without treatment may be reasonable in cases with no ulcerations or bothersome symptoms, considering that spontaneous resolution can occur in up to 17% of lesions.
However, in certain situations, intervention may be necessary. Compression therapy can be beneficial for managing edema and facilitating healing, especially in individuals with concurrent venous disease or lymphedema. When ulcerations are present, proper wound care principles are crucial.
First-line treatments for NL include potent topical corticosteroids for early lesions and intralesional corticosteroid injections into the active borders of established lesions. Topical steroids should be avoided in inactive, atrophic lesions as they can worsen atrophy and increase the risk of new ulcerations.
Antiplatelet aggregation therapy has been attempted in some cases using medications such as dipyridamole and aspirin. This approach is based on the theory that NL may involve platelet-mediated vascular occlusion or altered platelet survival. However, the effectiveness of this therapy is not well-established.
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Necrobiosis lipoidica (NL) is an infrequent, long-lasting, and unexplained granulomatous condition characterized by collagen degeneration. It carries an intensified risk of ulceration and is commonly linked to type 1 diabetes mellitus. The disease is characterized by thickened blood vessel walls and the accumulation of fat.
One of its primary complications is the development of ulcers, typically triggered by physical injury. Infections can also occur, although they are less common. Additionally, in rare cases, if necrobiosis lipoidica persists over time, it may transform into squamous cell carcinoma.
Necrobiosis lipoidica is more commonly observed in individuals with diabetes, although the validity of this association is currently subject to debate. The incidence of necrobiosis lipoidica among people with diabetes ranges from 0.3% to 1.2%. It is important to note that necrobiosis lipoidica may occur before the onset of diabetes in approximately 14% of cases, simultaneously with diabetes in around 24% of cases and after the diagnosis of diabetes in 62% of cases.
The likelihood of developing necrobiosis lipoidica is not influenced by the degree of glycemic control, as there is no proven correlation. There is a higher prevalence of necrobiosis lipoidica in females, accounting for approximately 77% of cases, and the onset of the condition tends to occur at a younger age in women compared to men. The average age of onset for necrobiosis lipoidica is typically between 30 and 40 years.
One key aspect of necrobiosis lipoidica is collagen degeneration, specifically in the deep dermis and subcutaneous fat layer. The exact reason for this collagen degeneration is unclear, but it is thought to involve immune-mediated processes and abnormal inflammation. It is believed that immune dysfunction plays a role in the pathogenesis of NL. Inflammatory cells, such as T lymphocytes, macrophages, and neutrophils, infiltrate the affected skin. These immune cells release various inflammatory mediators, including cytokines and chemokines, contributing to tissue damage and inflammation.
Vascular changes are also observed in NL. The walls of small blood vessels thicken, leading to impaired blood flow and reduced oxygen supply to the affected area. This vascular abnormality may further contribute to tissue damage and the formation of characteristic lesions. The lipids accumulation in the affected skin is another characteristic feature of NL. It is believed to result from the impaired clearance of lipids due to vascular abnormalities and inflammation.
The exact role of lipid accumulation in the pathophysiology of NL is not fully understood, but it may contribute to tissue damage and inflammation. While there is an increased prevalence of NL in individuals with diabetes, the exact relationship between the two conditions remains uncertain. It is hypothesized that hyperglycemia, immune dysregulation, and vascular abnormalities associated with diabetes may play a role in the development or progression of NL in susceptible individuals.
The etiology of necrobiosis lipoidica remains uncertain and is considered multifactorial. While the exact cause is unknown, several factors have been proposed to contribute to the development of this condition.
Immunological Factors: Immune dysfunction and abnormal immune responses are believed to play a role in the pathogenesis of NL. There is evidence of T-cell-mediated immune reactions and an increased presence of various immune cells, including T lymphocytes, macrophages, and neutrophils, within the affected skin. These immune cells release inflammatory mediators that contribute to tissue damage and inflammation.
Vascular Abnormalities: Changes in the blood vessels supplying the affected skin have been observed in NL. Thickening of the vessel walls, narrowing of the lumen, and reduced blood flow may contribute to tissue hypoxia and damage.
Collagen Degeneration: NL is characterized by the degeneration of collagen in the deep dermis and subcutaneous fat layer. The reasons for this collagen degeneration are not well-defined, but it is believed to involve alterations in collagen synthesis and degradation processes.
Genetic Predisposition: There may be a genetic susceptibility to developing NL, as some cases have been reported in families. However, specific genes or genetic mutations associated with NL have not been identified.
Association with Diabetes Mellitus: Although the relationship is not fully understood, NL is more commonly seen in individuals with diabetes, particularly type 1 diabetes. The exact mechanisms linking NL and diabetes are unclear but may involve factors such as hyperglycemia, microvascular changes, and immune dysregulation associated with diabetes.
Trauma or Injury: Trauma or injury to the skin has been reported as a triggering factor for the development of NL lesions. However, not all cases of NL are associated with preceding trauma.
Clinical History
Necrobiosis lipoidica typically presents as well-defined, round, or oval-shaped plaques on the skin. These lesions are usually located on the lower legs but can also occur in other body areas. A yellowish or reddish-brown color characterizes the plaques, which may appear shiny or atrophic. The surface of the lesions may be smooth or slightly raised. The size and distribution of the lesions can vary.
They may range from a few millimeters to several centimeters in diameter. In some cases, multiple plaques may be present, and they can coalesce to form larger areas of involvement. Patients with necrobiosis lipoidica may experience various symptoms, although some individuals may remain asymptomatic. Common symptoms include itching, burning, and pain in the affected areas.
These symptoms can vary in intensity and may be aggravated by certain triggers. The lesions’ duration and progression over time are important to assess. Necrobiosis lipoidica can be a chronic condition with periods of stability or intermittent flare-ups. Documenting the history of lesion development and changes in size, color, or symptoms can provide valuable information.
Physical Examination
The primary characteristic of NL is the presence of well-defined plaques on the skin. These plaques typically have a yellowish or reddish-brown color and may appear shiny or atrophic. They can range from a few millimeters to several centimeters in diameter. The lesions are commonly located on the lower legs but can also occur in other areas of the body.
The texture of the lesions can vary. In some cases, the plaques may have a smooth surface, while in others, they may appear slightly raised or wrinkled. Over time, the lesions may become atrophic, meaning the skin in the affected area appears thin and depressed.
This atrophy can contribute to the glossy appearance of the plaques. In some cases, NL plaques may develop ulcers, especially following trauma or injury to the affected skin. These ulcers can be deep, slow to heal, and may increase the risk of infection. The skin surrounding the NL lesions may show signs of inflammation, such as erythema or edema. The patient may report itching, burning, or pain in the NL lesions.
Differential Diagnoses
Xanthomas
Rheumatoid Arthritis
Sarcoidosis
Granuloma Annulare
The necrobiosis lipoidica treatment remains challenging, as no definitive therapy has been proven effective. Glycemic control in patients with diabetes mellitus does not typically significantly impact the course of NL. A conservative approach without treatment may be reasonable in cases with no ulcerations or bothersome symptoms, considering that spontaneous resolution can occur in up to 17% of lesions.
However, in certain situations, intervention may be necessary. Compression therapy can be beneficial for managing edema and facilitating healing, especially in individuals with concurrent venous disease or lymphedema. When ulcerations are present, proper wound care principles are crucial.
First-line treatments for NL include potent topical corticosteroids for early lesions and intralesional corticosteroid injections into the active borders of established lesions. Topical steroids should be avoided in inactive, atrophic lesions as they can worsen atrophy and increase the risk of new ulcerations.
Antiplatelet aggregation therapy has been attempted in some cases using medications such as dipyridamole and aspirin. This approach is based on the theory that NL may involve platelet-mediated vascular occlusion or altered platelet survival. However, the effectiveness of this therapy is not well-established.
medtigo
Necrobiosis Lipoidica
Updated :
August 24, 2023
Necrobiosis lipoidica (NL) is an infrequent, long-lasting, and unexplained granulomatous condition characterized by collagen degeneration. It carries an intensified risk of ulceration and is commonly linked to type 1 diabetes mellitus. The disease is characterized by thickened blood vessel walls and the accumulation of fat.
One of its primary complications is the development of ulcers, typically triggered by physical injury. Infections can also occur, although they are less common. Additionally, in rare cases, if necrobiosis lipoidica persists over time, it may transform into squamous cell carcinoma.
Necrobiosis lipoidica is more commonly observed in individuals with diabetes, although the validity of this association is currently subject to debate. The incidence of necrobiosis lipoidica among people with diabetes ranges from 0.3% to 1.2%. It is important to note that necrobiosis lipoidica may occur before the onset of diabetes in approximately 14% of cases, simultaneously with diabetes in around 24% of cases and after the diagnosis of diabetes in 62% of cases.
The likelihood of developing necrobiosis lipoidica is not influenced by the degree of glycemic control, as there is no proven correlation. There is a higher prevalence of necrobiosis lipoidica in females, accounting for approximately 77% of cases, and the onset of the condition tends to occur at a younger age in women compared to men. The average age of onset for necrobiosis lipoidica is typically between 30 and 40 years.
One key aspect of necrobiosis lipoidica is collagen degeneration, specifically in the deep dermis and subcutaneous fat layer. The exact reason for this collagen degeneration is unclear, but it is thought to involve immune-mediated processes and abnormal inflammation. It is believed that immune dysfunction plays a role in the pathogenesis of NL. Inflammatory cells, such as T lymphocytes, macrophages, and neutrophils, infiltrate the affected skin. These immune cells release various inflammatory mediators, including cytokines and chemokines, contributing to tissue damage and inflammation.
Vascular changes are also observed in NL. The walls of small blood vessels thicken, leading to impaired blood flow and reduced oxygen supply to the affected area. This vascular abnormality may further contribute to tissue damage and the formation of characteristic lesions. The lipids accumulation in the affected skin is another characteristic feature of NL. It is believed to result from the impaired clearance of lipids due to vascular abnormalities and inflammation.
The exact role of lipid accumulation in the pathophysiology of NL is not fully understood, but it may contribute to tissue damage and inflammation. While there is an increased prevalence of NL in individuals with diabetes, the exact relationship between the two conditions remains uncertain. It is hypothesized that hyperglycemia, immune dysregulation, and vascular abnormalities associated with diabetes may play a role in the development or progression of NL in susceptible individuals.
The etiology of necrobiosis lipoidica remains uncertain and is considered multifactorial. While the exact cause is unknown, several factors have been proposed to contribute to the development of this condition.
Immunological Factors: Immune dysfunction and abnormal immune responses are believed to play a role in the pathogenesis of NL. There is evidence of T-cell-mediated immune reactions and an increased presence of various immune cells, including T lymphocytes, macrophages, and neutrophils, within the affected skin. These immune cells release inflammatory mediators that contribute to tissue damage and inflammation.
Vascular Abnormalities: Changes in the blood vessels supplying the affected skin have been observed in NL. Thickening of the vessel walls, narrowing of the lumen, and reduced blood flow may contribute to tissue hypoxia and damage.
Collagen Degeneration: NL is characterized by the degeneration of collagen in the deep dermis and subcutaneous fat layer. The reasons for this collagen degeneration are not well-defined, but it is believed to involve alterations in collagen synthesis and degradation processes.
Genetic Predisposition: There may be a genetic susceptibility to developing NL, as some cases have been reported in families. However, specific genes or genetic mutations associated with NL have not been identified.
Association with Diabetes Mellitus: Although the relationship is not fully understood, NL is more commonly seen in individuals with diabetes, particularly type 1 diabetes. The exact mechanisms linking NL and diabetes are unclear but may involve factors such as hyperglycemia, microvascular changes, and immune dysregulation associated with diabetes.
Trauma or Injury: Trauma or injury to the skin has been reported as a triggering factor for the development of NL lesions. However, not all cases of NL are associated with preceding trauma.
Clinical History
Necrobiosis lipoidica typically presents as well-defined, round, or oval-shaped plaques on the skin. These lesions are usually located on the lower legs but can also occur in other body areas. A yellowish or reddish-brown color characterizes the plaques, which may appear shiny or atrophic. The surface of the lesions may be smooth or slightly raised. The size and distribution of the lesions can vary.
They may range from a few millimeters to several centimeters in diameter. In some cases, multiple plaques may be present, and they can coalesce to form larger areas of involvement. Patients with necrobiosis lipoidica may experience various symptoms, although some individuals may remain asymptomatic. Common symptoms include itching, burning, and pain in the affected areas.
These symptoms can vary in intensity and may be aggravated by certain triggers. The lesions’ duration and progression over time are important to assess. Necrobiosis lipoidica can be a chronic condition with periods of stability or intermittent flare-ups. Documenting the history of lesion development and changes in size, color, or symptoms can provide valuable information.
Physical Examination
The primary characteristic of NL is the presence of well-defined plaques on the skin. These plaques typically have a yellowish or reddish-brown color and may appear shiny or atrophic. They can range from a few millimeters to several centimeters in diameter. The lesions are commonly located on the lower legs but can also occur in other areas of the body.
The texture of the lesions can vary. In some cases, the plaques may have a smooth surface, while in others, they may appear slightly raised or wrinkled. Over time, the lesions may become atrophic, meaning the skin in the affected area appears thin and depressed.
This atrophy can contribute to the glossy appearance of the plaques. In some cases, NL plaques may develop ulcers, especially following trauma or injury to the affected skin. These ulcers can be deep, slow to heal, and may increase the risk of infection. The skin surrounding the NL lesions may show signs of inflammation, such as erythema or edema. The patient may report itching, burning, or pain in the NL lesions.
Differential Diagnoses
Xanthomas
Rheumatoid Arthritis
Sarcoidosis
Granuloma Annulare
The necrobiosis lipoidica treatment remains challenging, as no definitive therapy has been proven effective. Glycemic control in patients with diabetes mellitus does not typically significantly impact the course of NL. A conservative approach without treatment may be reasonable in cases with no ulcerations or bothersome symptoms, considering that spontaneous resolution can occur in up to 17% of lesions.
However, in certain situations, intervention may be necessary. Compression therapy can be beneficial for managing edema and facilitating healing, especially in individuals with concurrent venous disease or lymphedema. When ulcerations are present, proper wound care principles are crucial.
First-line treatments for NL include potent topical corticosteroids for early lesions and intralesional corticosteroid injections into the active borders of established lesions. Topical steroids should be avoided in inactive, atrophic lesions as they can worsen atrophy and increase the risk of new ulcerations.
Antiplatelet aggregation therapy has been attempted in some cases using medications such as dipyridamole and aspirin. This approach is based on the theory that NL may involve platelet-mediated vascular occlusion or altered platelet survival. However, the effectiveness of this therapy is not well-established.
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