Using baricitinib to treat Kohlmeier-Degos disease with neurological symptoms

A Phase IIa Trial of Baricitinib in the Treatment of Kohlmeier-Degos Disease Patients With Neurological Involvement

PHASE2 · National Institutes of Health Clinical Center (CC) · NCT06923072

This study is testing if the medication baricitinib can help adults with Kohlmeier-Degos disease who have neurological symptoms feel better over 24 weeks.

Quick facts

PhasePHASE2
Study typeInterventional
Enrollment12 (estimated)
Ages18 Years to 99 Years
SexAll
SponsorNational Institutes of Health Clinical Center (CC) (nih)
Drugs / interventionsbaricitinib
Locations1 site (Bethesda, Maryland)
Trial IDNCT06923072 on ClinicalTrials.gov

What this trial studies

This phase II clinical trial aims to evaluate the efficacy of baricitinib, an FDA-approved medication, in patients suffering from Kohlmeier-Degos disease (KD) who exhibit neurological involvement. Participants aged 18 and older will undergo a series of evaluations, including imaging and lumbar punctures, before receiving baricitinib for 24 weeks, alongside their usual medications. The study will assess the progression of neurological symptoms and patient-reported outcomes over a total of 40 weeks. The hypothesis is that baricitinib will reduce disease progression by targeting specific immune signaling pathways.

Who should consider this trial

Good fit: Ideal candidates are adults aged 18 and older diagnosed with Kohlmeier-Degos disease who have neurological abnormalities.

Not a fit: Patients with active infections or those unable to comply with the study procedures may not benefit from this trial.

Why it matters

Potential benefit: If successful, this treatment could significantly improve the management of neurological symptoms in patients with Kohlmeier-Degos disease.

How similar studies have performed: While baricitinib has been effective in other conditions, this specific application for Kohlmeier-Degos disease is novel and has not been extensively tested.

Eligibility criteria

Show full inclusion / exclusion criteria
* 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

Where this trial is running

Bethesda, Maryland

Study contacts

How to participate

  1. Review the eligibility criteria above with your treating physician.
  2. Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
  3. Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.

View on ClinicalTrials.gov →

Conditions: Kohlmeier-Degos Disease, Malignant Atrophic Papulosis, Degos Disease, Papulosis, Malignant Atrophic, Kohlmeier-Degos, Baricitinib, Neurological involvement of Degos Disease

Last reviewed 2026-05-15 by the Find a Trial editorial team. Information on this page is for educational purposes and is not medical advice. Always consult qualified healthcare professionals about clinical trial participation.