Baricitinib in the Treatment of Kohlmeier-Degos Disease in Patients With Neurological Involvement

Recruiting now · Phase 2

Conditions studied: Kohlmeier-Degos Disease, Malignant Atrophic Papulosis, Degos Disease, Papulosis, Malignant Atrophic

In brief

Background: Kohlmeier-Degos (KD) is a rare disease that causes inflammation and blood clots, leading to blockages in small blood vessels. These blockages can result in K-D lesions throughout the body, affecting the skin, lungs, heart, spinal cord, and brain. KD can be fatal. No treatment exists for this disease. Objective: To test a study drug (baricitinib) in people with brain and spine lesions caused by KD disease. Baricitinib is FDA approved to treat other disorders but has not yet been tried in people with KD. Eligibility: People aged 18 years or older with KD-related lesions in the brain and spine. Design: Participants will be screened; they will have a physical exam with blood tests. They will also have a baseline visit that may include multiple tests, such as imaging scans of the brain and spine; a lumbar puncture to collect fluid from the spinal canal; and a meeting with a neurologist. They will fill out a questionnaire about their health. They will continue to take their normal medications throughout the study. Baricitinib is a tablet taken by mouth. Participants will remain on their normal medications for 12 weeks after their baseline visit. Then they will also take the study drug once a day at home for 24 weeks. Participants will have clinic visits every few weeks for up to 40 weeks. Some visits may take 1 to 4 days. Baseline tests will be repeated 3 more times during study visits. Other visits will require only blood tests; these may be done by local labs that will send the samples to NIH; 2 visits may be done via telehealth....

Key facts

Study ID: NCT06923072
Run by: National Heart, Lung, and Blood Institute (NHLBI)
People needed: 12
Starts: 2026-02-18
Expected to finish: 2030-12-15
Last updated by the study team: 2026-06-22

Who can join

Age: 18 and older, up to 99. Sex: any. Healthy volunteers: not accepted.

You may not qualify if…

Active infection not responding to appropriate therapy
Hemoglobin <7 g/dL
Platelet counts < 50 K /mcL
Neutropenia (ANC <0.5 x k/mcL)
Lymphopenia (Absolute Lymphocyte Count [ALC] <0.2x k/mcL)
Liver function tests (LFTs > 2x time upper limit of normal)
Estimated Glomerular Filtration Rate (eGFR)/Creatinine (Cr < 30 mL/min)
Have experienced any of the following within 12 weeks of screening: VTE (DVT/pulmonary embolism [PE]), myocardial infarction (MI), unstable ischemic heart disease, stroke, or New York Heart Association Stage III/IV heart failure.
Have had symptomatic herpes zoster infection within 12 weeks prior to a enrolling in the study .
Have had household contact with a person with active TB and did not receive appropriate and documented prophylaxis for TB.
Have evidence of active TB or latent TB
Have been exposed to a live vaccine within 12 weeks of baricitinib treatment or are expected to need/receive a live vaccine during the course of the study
No gadolinium based contrast agent exposure is permitted if eGFR < 30 mL/min/1.73m\^2 using the CKD-EPI equation measured within 5 days .
Breast feeding
Pregnancy
Uncontrolled malignancy

Where it is running

National Institutes of Health Clinical Center — Bethesda, Maryland, United States (enrolling)

Full record on ClinicalTrials.gov

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