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NCT05998395

JAK/STAT Inhibition in CNS Kohlmeier-Degos Disease

Completed Phase 1, PHASE2 Results posted Last updated 3 February 2026
What this trial tests

Phase 1, PHASE2 trial testing Ruxolitinib in Kohlmeier Degos Disease With Neurologic Involvement in 1 participant. Completed in 28 February 2025.

Timeline
28 September 2023
Primary endpoint
28 February 2025
28 February 2025

Quick facts

Lead sponsorNational Heart, Lung, and Blood Institute (NHLBI)
PhasePhase 1, PHASE2
StatusCompleted
Study typeINTERVENTIONAL
Allocationna
Designsingle group
Maskingnone
Primary purposetreatment
Enrollment1
Start date28 September 2023
Primary completion28 February 2025
Estimated completion28 February 2025
Sites1 location across United States

Drugs / interventions tested

Conditions studied

Sponsor

National Heart, Lung, and Blood Institute (NHLBI)

Who can join

Adults 18 to 100, any sex, with Kohlmeier Degos Disease With Neurologic Involvement or Kohlmeier-Degos Disease. Patients with the condition only — healthy volunteers not accepted.

Results — posted to ClinicalTrials.gov

Per-arm endpoint measurements with 95% confidence intervals where reported. Source: trial results section.

Number of Participants With Stable or no Lesions Developed Based on MRI Neurological Involvement of K-D Disease Following Ruloxitinib Primary · Baseline, up to 13 weeks

The primary endpoint is stability or regression of existing enhancing lesions or no development of new enhancing lesions in the brain and spine observed in MRI evaluation after 13 weeks and up to 73 weeks of ruloxitinib (10 mg BID) compared to pre-treatment MRI images.

GroupValue95% CI
Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease1
Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease0
Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease0
Number of Participants With Stable or no Lesions Developed Based on MRI Neurological Involvement of K-D Disease Following Ruloxitinib Primary · 13 weeks up to 72 weeks

The primary endpoint is stability or regression of existing enhancing lesions or no development of new enhancing lesions in the brain and spine observed in MRI evaluation after 13 weeks and up to 73 weeks of ruloxitinib (10 mg BID) compared to pre-treatment MRI images.

GroupValue95% CI
Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease0
Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease0
Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease1

Adverse events — posted to ClinicalTrials.gov

Time frame: Up to 72 weeks. Reporting threshold: 0%. Adverse-event reports describe events observed during the trial — not all are caused by the drug.

Ruxolitinib in Participants With CNS Kohlmeier-Degos Disease
Serious: 1/1 (100%)
Deaths: 0/1

Serious adverse events (4 terms)

ReactionSystemRuxolitinib in Participant…
Worsening Sacral wound abscessSkin and subcutaneous tissue disorders
AnemiaBlood and lymphatic system disorders
BacteremiaInfections and infestations
SepsisInfections and infestations
Other adverse events (2 terms — click to expand)

ReactionSystemRuxolitinib in Participant…
DepressionPsychiatric disorders
Abnormal MRIGeneral disorders

Most-reported serious reactions: Worsening Sacral wound abscess, Anemia, Bacteremia, Sepsis.

Data from ClinicalTrials.gov NCT05998395 adverse events section.

Sponsor's own description

Background: Kohlmeier-Degos (K-D) is a rare disease that leads to the inflammation and/or blockage of small blood vessels in many organs; these can include the skin, eyes (rare), small bowels, lungs, heart, and the brain and spinal cord (central nervous system, or CNS). There are no known effective treatments for K-D that affects the CNS. Objective: To test a drug (ruxolitinib) in a person with K-D affecting the CNS. Eligibility: This study is designed to treat 1 adult participant with K-D affecting the CNS. Design: The participant will be screened: They will have a physical exam and blood tests. They will have skin biopsies: Small samples of skin will be removed. They will have a lumbar puncture: A needle will be inserted in his back to draw fluid from the space around the spinal cord. They will have a magnetic resonance imaging (MRI) scan: they will lie on a table that slides into a tube to take pictures of their brain and spinal cord. They will see a doctor who specializes in nerves. Ruxolitinib is a tablet taken by mouth. The participant will take the drug twice a day for up to 26 weeks. The dosage may change over time. The participant will have up to 7 clinic visits in 28 weeks. Each visit will be 1 to 3 days. MRI scans, biopsies, lumbar punctures, and other blood tests will be repeated on different visits. The participant may receive follow-up phone calls between visits. The participant will report any adverse effects. Unscheduled visits may be needed if new symptoms develop. The last follow-up will be 4 weeks after the last dose of the study drug.

Publications & conference data

No peer-reviewed publications indexed yet for this trial. Completed trials usually publish results within 12-18 months.

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Other trials of Ruxolitinib

Trials testing the same drug.

Other National Heart, Lung, and Blood Institute (NHLBI) trials

Trials by the same sponsor.

Verify against primary sources

Data sources for this page

Drug Landscape aggregates and links these public records for informational use only. Always verify against the primary source before clinical or regulatory decisions. Canonical URL: https://druglandscape.com/trial/NCT05998395.

Primary sources · FDA · ClinicalTrials.gov · EMA · SEC EDGAR · ChEMBL · Wikidata · full sourcing