ICD10 code of Degos Disease and ICD9 code

Degos disease, also known as malignant atrophic papulosis, does not have a unique, dedicated code in the ICD-10 or ICD-9 systems; it is typically classified under broader categories for vasculitis or skin disorders. Clinicians often use ICD-10 code L94.5 (Poikiloderma vasculare atrophicans) or ICD-9 code 701.8 (Other specified hypertrophic and atrophic conditions of skin) for administrative and billing purposes.

What is the clinical nature of Degos disease?

Degos disease is an exceptionally rare, progressive vasculopathy characterized by occlusive lesions of small-to-medium-sized arteries. These lesions lead to tissue infarction, most notably manifesting as pathognomonic porcelain-white skin papules with erythematous borders. While the skin involvement is the most visible sign, Degos disease can involve systemic manifestations, primarily affecting the gastrointestinal tract and the central nervous system due to vessel occlusion.

How is Degos disease classified for medical coding?

Because Degos disease is so rare—with fewer than 200 cases reported in the literature—it lacks a specific diagnostic code. Healthcare providers must utilize "catch-all" codes. When coding for Degos disease, the choice often depends on the primary presenting symptoms:

• ICD-10 L94.5: Often used for the cutaneous manifestations of Degos disease.


• ICD-10 I77.6: Sometimes utilized if the physician focuses on the underlying arteritis or vascular occlusion.


• ICD-9 701.8: The historical code frequently associated with the condition in older medical records.

Is Degos disease common?

Degos disease is considered an ultra-rare condition. At DiseaseMaps.org, we currently have 18 members who have shared their experiences living with this diagnosis, highlighting the rarity and the isolation often felt by patients. Understanding that Degos disease has no dedicated code is a common source of frustration for patients navigating insurance and disability claims.

Next steps

• Consult with a rheumatologist or dermatologist experienced in systemic vasculitis to manage Degos disease symptoms.


• Connect with the 18 other members on DiseaseMaps.org to share insights on navigating healthcare systems with a rare diagnosis.


• Request that your specialist documents the condition as "Degos Disease (Malignant Atrophic Papulosis)" in the clinical notes, regardless of the billing code used.

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.

References

• Orphanet: Malignant atrophic papulosis (ORPHA:2356).


• NIH GARD: Degos disease information page.


• OMIM (Online Mendelian Inheritance in Man): Entry #124150.


• Journal of the American Academy of Dermatology: Recent reviews on the systemic management of Degos disease.