Short answer · Medically reviewed summary · Last updated: 2026-05-08
Degos disease, also known as malignant atrophic papulosis, is a rare systemic vasculopathy characterized by occlusive lesions of small-to-medium-sized arteries. While "Degos disease" is the most common clinical identifier, the condition is also frequently referred to as Köhlmeier-Degos disease or by the descriptive term malignant atrophic papulosis to reflect its historical and clinical development. What are the historical and alternative names for Degos disease? The nomenclature for Degos disease has evolved since its initial description in the early 20th century.
Degos Disease synonyms
Other names for Degos Disease: synonyms, acronyms and related terms used by doctors and patients.
Degos disease, also known as malignant atrophic papulosis, is a rare systemic vasculopathy characterized by occlusive lesions of small-to-medium-sized arteries. While "Degos disease" is the most common clinical identifier, the condition is also frequently referred to as Köhlmeier-Degos disease or by the descriptive term malignant atrophic papulosis to reflect its historical and clinical development.
What are the historical and alternative names for Degos disease?
The nomenclature for Degos disease has evolved since its initial description in the early 20th century. Historical literature often refers to the condition as Köhlmeier-Degos disease, named after the two physicians who independently described the clinical findings. You may also encounter the term "malignant atrophic papulosis" (MAP), which describes the hallmark skin lesions. In some older European medical texts, the condition was occasionally documented as thrombo-angiitis cutanea et visceralis, though this term is rarely used in modern clinical practice.
How is Degos disease classified in medical systems?
Medical professionals and researchers categorize Degos disease under specific coding systems to ensure accurate documentation and research tracking. The following identifiers are commonly used in international databases:
- Orphanet: Classified as ORPHA2281, identifying it as a rare systemic vasculopathy.
- OMIM: Listed under #131500, noting the clinical and genetic associations.
- ICD-10/11: Often categorized under codes relating to "other specified vasculopathies" or "malignant atrophic papulosis."
Why does Degos disease have multiple names?
The existence of multiple names for Degos disease stems from the historical practice of naming rare conditions after the clinicians who first identified them, alongside later efforts to create descriptive, pathological terminology. Because Degos disease involves both skin-limited and systemic forms, researchers have sometimes used different names to distinguish between the cutaneous-only presentation and the more severe, life-threatening systemic involvement of Degos disease. Today, Degos disease remains the preferred term in clinical settings to maintain consistency across global patient registries, including our own group of 18 members on DiseaseMaps.org who share insights on their diagnostic journeys.
Next steps
- Consult a board-certified dermatologist or rheumatologist experienced in rare vasculopathies.
- Request a skin biopsy to confirm the characteristic vascular occlusions associated with Degos disease.
- Connect with the 18 members on DiseaseMaps.org to share experiences and find peer support.
- Review the latest clinical trial registries at ClinicalTrials.gov for emerging treatments.
Medical disclaimer: This content is for informational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Degos disease overview.
- Orphanet: Malignant atrophic papulosis (ORPHA2281).
- OMIM (Online Mendelian Inheritance in Man): Entry #131500.
- PubMed/NCBI: Current literature on systemic vasculopathies and rare dermatological conditions.
