Status:
RECRUITING
Baricitinib in the Treatment of Kohlmeier-Degos Disease in Patients With Neurological Involvement
Lead Sponsor:
National Heart, Lung, and Blood Institute (NHLBI)
Conditions:
Kohlmeier-Degos Disease
Malignant Atrophic Papulosis
Eligibility:
All Genders
18-99 years
Phase:
PHASE2
Brief Summary
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...
Detailed Description
Study Description: This phase II study will provide off-label baricitinib treatment in patients with Kohlmeier Degos disease (K-D) with neurologic involvement. We will perform a baseline research eva...
Eligibility Criteria
Inclusion
- INCLUSION CRITERIA:
- In order to be eligible to participate in this study, an individual must meet all of the following criteria:
- Provision of signed and dated informed consent form by the subject or Legally Authorized Representative (LAR).
- Stated willingness to comply with all study procedures and availability for the duration of the study.
- Male or female, aged 18 or older.
- Subjects diagnosed with systemic Degos disease, who manifest neurologic abnormalities observed clinically, radiologically or in abnormal laboratory findings.
- Ability to take oral medication and be willing to adhere to the baricitinib regimen.
- For female patients of reproductive potential, non-pregnant, non-breastfeeding: agree to use of highly effective contraception for the duration of the study and 30 days after the last dose.
- Ability of subject or LAR to understand and the willingness to sign a written informed consent document.
- EXCLUSION CRITERIA:
- 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
Exclusion
Key Trial Info
Start Date :
January 14 2026
Trial Type :
INTERVENTIONAL
Allocation :
ESTIMATED
End Date :
December 15 2030
Estimated Enrollment :
12 Patients enrolled
Trial Details
Trial ID
NCT06923072
Start Date
January 14 2026
End Date
December 15 2030
Last Update
January 9 2026
Active Locations (1)
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1
National Institutes of Health Clinical Center
Bethesda, Maryland, United States, 20892