Degos disease

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Degos disease
Degos disease skin lesions
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

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

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

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

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 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

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

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
• Autoimmune disease
• Dermatology