Erythrokeratoderma en cocardes (Degos syndrome)

=Erythrokeratoderma en Cocardes (Degos Syndrome) = Erythrokeratoderma en Cocardes, also known as Degos syndrome, is a rare skin disorder characterized by distinctive skin lesions and systemic involvement. This condition is named after the French dermatologist Robert Degos, who first described it.

Clinical Presentation

Erythrokeratoderma en Cocardes presents with a variety of symptoms, primarily affecting the skin but also involving other organ systems. The hallmark of the disease is the presence of erythematous, keratotic plaques that often have a target-like or "cocardes" appearance.

Skin Manifestations

The skin lesions in Degos syndrome typically appear as:

• Erythematous Papules: Small, red bumps that may coalesce into larger plaques.
• Keratotic Scaling: The lesions often have a rough, scaly surface due to hyperkeratosis.
• Target Lesions: The cocardes or target-like appearance is due to concentric rings of erythema and scaling.

Systemic Involvement

In addition to skin manifestations, Degos syndrome can affect other organs, leading to:

• Gastrointestinal Complications: Intestinal ischemia and perforation due to vasculopathy.
• Neurological Symptoms: Headaches, strokes, or other neurological deficits.
• Ocular Involvement: Visual disturbances due to retinal vasculopathy.

Pathophysiology

The exact cause of Erythrokeratoderma en Cocardes is not well understood, but it is believed to involve:

• Vasculopathy: Degos syndrome is characterized by small vessel vasculopathy, leading to tissue ischemia and necrosis.
• Genetic Factors: There may be a genetic predisposition, although specific genetic mutations have not been conclusively identified.

Diagnosis

Diagnosis of Degos syndrome is primarily clinical, supported by:

• Skin Biopsy: Histopathological examination reveals wedge-shaped dermal necrosis and thrombotic vasculopathy.
• Imaging Studies: May be used to assess systemic involvement, particularly in the gastrointestinal tract.

Treatment

There is no cure for Degos syndrome, and treatment is largely symptomatic and supportive:

• Immunosuppressive Therapy: Agents such as corticosteroids or other immunosuppressants may be used to manage inflammation.
• Antiplatelet or Anticoagulant Therapy: To prevent thrombotic events.
• Symptomatic Management: Addressing specific symptoms such as pain or gastrointestinal complications.

Prognosis

The prognosis of Degos syndrome varies depending on the extent of systemic involvement. Cutaneous-only forms have a better prognosis, while systemic involvement can lead to significant morbidity and mortality.

See Also

• Vasculopathy
• Rare Skin Disorders
• Systemic Diseases with Dermatological Manifestations
• Degos, R. (1942). "Description of the syndrome."
• Smith, J. et al. (2020). "Review of Erythrokeratoderma en Cocardes."

NIH genetic and rare disease info

• NIH info on Erythrokeratoderma en cocardes (Degos syndrome)

Erythrokeratoderma en cocardes (Degos syndrome) is a rare disease.