Degos disease: Difference between revisions
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{{Infobox medical condition | |||
| name = Degos disease | |||
| image = [[File:Degos-disease.jpg|left|thumb|Degos disease skin lesions]] | |||
| caption = Skin lesions characteristic of Degos disease | |||
| synonyms = Malignant atrophic papulosis | |||
| specialty = [[Dermatology]], [[Rheumatology]] | |||
| symptoms = [[Skin lesions]], [[abdominal pain]], [[neurological symptoms]] | |||
| complications = [[Bowel perforation]], [[stroke]], [[myocardial infarction]] | |||
| onset = Typically in [[adulthood]] | |||
| duration = Chronic | |||
| causes = Unknown | |||
| risks = Possible [[genetic predisposition]] | |||
| diagnosis = [[Clinical diagnosis]], [[skin biopsy]] | |||
| differential = [[Lupus erythematosus]], [[scleroderma]], [[vasculitis]] | |||
| treatment = [[Anticoagulants]], [[immunosuppressants]], [[plasmapheresis]] | |||
| prognosis = Variable; can be [[fatal]] if systemic | |||
| frequency = Rare | |||
}} | |||
{{Short description|A rare vasculopathy affecting the skin and internal organs}} | {{Short description|A rare vasculopathy affecting the skin and internal organs}} | ||
'''Degos disease''', also known as '''malignant atrophic papulosis''', is a rare [[vasculopathy]] characterized by distinctive skin lesions and potential involvement of internal organs. The disease is named after the French dermatologist [[Robert Degos]], who first described it in 1941. | '''Degos disease''', also known as '''malignant atrophic papulosis''', is a rare [[vasculopathy]] characterized by distinctive skin lesions and potential involvement of internal organs. The disease is named after the French dermatologist [[Robert Degos]], who first described it in 1941. | ||
==Clinical presentation== | ==Clinical presentation== | ||
Degos disease typically presents with small, erythematous papules that evolve into porcelain-white atrophic scars with a surrounding telangiectatic rim. These lesions are most commonly found on the trunk and extremities. The disease can be divided into two forms: a benign cutaneous form and a systemic form that involves internal organs. | Degos disease typically presents with small, erythematous papules that evolve into porcelain-white atrophic scars with a surrounding telangiectatic rim. These lesions are most commonly found on the trunk and extremities. The disease can be divided into two forms: a benign cutaneous form and a systemic form that involves internal organs. | ||
===Cutaneous manifestations=== | ===Cutaneous manifestations=== | ||
The cutaneous form of Degos disease is characterized by the presence of multiple papules that develop into atrophic scars. These lesions are pathognomonic and are often the first sign of the disease. The skin lesions are usually asymptomatic but can be cosmetically concerning for patients. | The cutaneous form of Degos disease is characterized by the presence of multiple papules that develop into atrophic scars. These lesions are pathognomonic and are often the first sign of the disease. The skin lesions are usually asymptomatic but can be cosmetically concerning for patients. | ||
===Systemic involvement=== | ===Systemic involvement=== | ||
In the systemic form of Degos disease, internal organs such as the gastrointestinal tract, central nervous system, and cardiovascular system may be affected. This can lead to serious complications, including bowel perforation, stroke, and myocardial infarction. The systemic form is associated with a poorer prognosis compared to the cutaneous form. | In the systemic form of Degos disease, internal organs such as the gastrointestinal tract, central nervous system, and cardiovascular system may be affected. This can lead to serious complications, including bowel perforation, stroke, and myocardial infarction. The systemic form is associated with a poorer prognosis compared to the cutaneous form. | ||
==Pathophysiology== | ==Pathophysiology== | ||
The exact cause of Degos disease is unknown, but it is believed to be an [[autoimmune]] process that leads to occlusion of small and medium-sized blood vessels. This results in tissue ischemia and the characteristic skin and organ lesions. Histopathological examination of skin lesions reveals wedge-shaped dermal necrosis and a lymphocytic infiltrate. | The exact cause of Degos disease is unknown, but it is believed to be an [[autoimmune]] process that leads to occlusion of small and medium-sized blood vessels. This results in tissue ischemia and the characteristic skin and organ lesions. Histopathological examination of skin lesions reveals wedge-shaped dermal necrosis and a lymphocytic infiltrate. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of Degos disease is primarily clinical, based on the characteristic skin lesions. A skin biopsy can confirm the diagnosis by demonstrating the typical histopathological features. Additional investigations may be required to assess for systemic involvement, including imaging studies and endoscopy. | Diagnosis of Degos disease is primarily clinical, based on the characteristic skin lesions. A skin biopsy can confirm the diagnosis by demonstrating the typical histopathological features. Additional investigations may be required to assess for systemic involvement, including imaging studies and endoscopy. | ||
==Management== | ==Management== | ||
There is no cure for Degos disease, and treatment is primarily supportive. Management focuses on alleviating symptoms and preventing complications. Antiplatelet agents, anticoagulants, and immunosuppressive therapies have been used with varying success. Regular monitoring for systemic involvement is crucial. | There is no cure for Degos disease, and treatment is primarily supportive. Management focuses on alleviating symptoms and preventing complications. Antiplatelet agents, anticoagulants, and immunosuppressive therapies have been used with varying success. Regular monitoring for systemic involvement is crucial. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis of Degos disease varies depending on the extent of systemic involvement. The cutaneous form generally has a benign course, while the systemic form can be life-threatening due to complications such as bowel perforation and stroke. | The prognosis of Degos disease varies depending on the extent of systemic involvement. The cutaneous form generally has a benign course, while the systemic form can be life-threatening due to complications such as bowel perforation and stroke. | ||
==See also== | |||
== | |||
* [[Vasculitis]] | * [[Vasculitis]] | ||
* [[Autoimmune disease]] | * [[Autoimmune disease]] | ||
* [[Dermatology]] | * [[Dermatology]] | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Autoimmune diseases]] | [[Category:Autoimmune diseases]] | ||
Latest revision as of 18:21, 5 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD's medical weight loss NYC, sleep center NYC
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| Degos disease | |
|---|---|
 | |
| Synonyms | Malignant atrophic papulosis |
| Pronounce | N/A |
| Specialty | Dermatology, Rheumatology |
| Symptoms | Skin lesions, abdominal pain, neurological symptoms |
| Complications | Bowel perforation, stroke, myocardial infarction |
| Onset | Typically in adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Unknown |
| Risks | Possible genetic predisposition |
| Diagnosis | Clinical diagnosis, skin biopsy |
| Differential diagnosis | Lupus erythematosus, scleroderma, vasculitis |
| Prevention | N/A |
| Treatment | Anticoagulants, immunosuppressants, plasmapheresis |
| Medication | N/A |
| Prognosis | Variable; can be fatal if systemic |
| Frequency | Rare |
| Deaths | N/A |
A rare vasculopathy affecting the skin and internal organs
Degos disease, also known as malignant atrophic papulosis, is a rare vasculopathy characterized by distinctive skin lesions and potential involvement of internal organs. The disease is named after the French dermatologist Robert Degos, who first described it in 1941.
Clinical presentation[edit]
Degos disease typically presents with small, erythematous papules that evolve into porcelain-white atrophic scars with a surrounding telangiectatic rim. These lesions are most commonly found on the trunk and extremities. The disease can be divided into two forms: a benign cutaneous form and a systemic form that involves internal organs.
Cutaneous manifestations[edit]
The cutaneous form of Degos disease is characterized by the presence of multiple papules that develop into atrophic scars. These lesions are pathognomonic and are often the first sign of the disease. The skin lesions are usually asymptomatic but can be cosmetically concerning for patients.
Systemic involvement[edit]
In the systemic form of Degos disease, internal organs such as the gastrointestinal tract, central nervous system, and cardiovascular system may be affected. This can lead to serious complications, including bowel perforation, stroke, and myocardial infarction. The systemic form is associated with a poorer prognosis compared to the cutaneous form.
Pathophysiology[edit]
The exact cause of Degos disease is unknown, but it is believed to be an autoimmune process that leads to occlusion of small and medium-sized blood vessels. This results in tissue ischemia and the characteristic skin and organ lesions. Histopathological examination of skin lesions reveals wedge-shaped dermal necrosis and a lymphocytic infiltrate.
Diagnosis[edit]
Diagnosis of Degos disease is primarily clinical, based on the characteristic skin lesions. A skin biopsy can confirm the diagnosis by demonstrating the typical histopathological features. Additional investigations may be required to assess for systemic involvement, including imaging studies and endoscopy.
Management[edit]
There is no cure for Degos disease, and treatment is primarily supportive. Management focuses on alleviating symptoms and preventing complications. Antiplatelet agents, anticoagulants, and immunosuppressive therapies have been used with varying success. Regular monitoring for systemic involvement is crucial.
Prognosis[edit]
The prognosis of Degos disease varies depending on the extent of systemic involvement. The cutaneous form generally has a benign course, while the systemic form can be life-threatening due to complications such as bowel perforation and stroke.
