Kohlmeier-Degos disease

Kohlmeier-Degos Disease Kohlmeier-Degos disease, also known as malignant atrophic papulosis, is a rare vasculopathy characterized by distinctive skin lesions and systemic involvement. The disease is named after the German dermatologist Kohlmeier, who first described it, and Degos, who further elaborated on its clinical features.

Clinical Presentation

Kohlmeier-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 also affect internal organs, leading to serious complications.

Skin Lesions

The hallmark of Kohlmeier-Degos disease is the skin lesions, which are initially red papules that develop into atrophic scars. These lesions are pathognomonic and are crucial for diagnosis.

Systemic Involvement

Systemic involvement can include:

• Gastrointestinal tract: Intestinal perforation and ischemia can occur, leading to severe abdominal pain and potentially life-threatening complications.
• Central nervous system: Neurological symptoms may arise due to cerebral vasculopathy, including headaches, seizures, and strokes.
• Other organs: The disease can also affect the heart, lungs, and kidneys, though less commonly.

Pathophysiology

The exact cause of Kohlmeier-Degos disease is unknown, but it is believed to be a small-vessel vasculopathy. Histopathological examination of skin lesions reveals a wedge-shaped area of dermal necrosis with fibrin deposition and thrombosis of small vessels.

Diagnosis

Diagnosis is primarily clinical, supported by histopathological findings from a skin biopsy. Differential diagnosis includes other vasculopathies and connective tissue diseases.

Treatment

There is no definitive cure for Kohlmeier-Degos disease. Treatment is symptomatic and supportive, focusing on managing complications. Options include:

• Antiplatelet agents: To reduce thrombotic events.
• Immunosuppressive therapy: Such as corticosteroids or other agents, though efficacy is variable.
• Surgical intervention: May be necessary for gastrointestinal complications.

Prognosis

The prognosis of Kohlmeier-Degos disease varies. Cutaneous-only forms have a better prognosis, while systemic involvement can lead to significant morbidity and mortality.

Epidemiology

Kohlmeier-Degos disease is extremely rare, with only a few hundred cases reported in the literature. It affects both genders and can occur at any age, though it is most commonly diagnosed in adults.

See Also

• Vasculopathy
• Connective tissue disease
• Skin biopsy
• Degos, R. (1942). "Malignant atrophic papulosis". *Journal of Dermatology*.
• Kohlmeier, W. (1941). "Initial description of the disease". *Dermatologische Wochenschrift*.

NIH genetic and rare disease info

• NIH info on Kohlmeier-Degos disease

Kohlmeier-Degos disease is a rare disease.