Efficacy of Guselkumab in Treating Hailey Hailey Disease
ConditionHailey Hailey Disease
Trial statusRecruiting
Trial phasePhase 2
Trial typeInterventional
Biological sexAll
Age18-90
SponsorYale University
About this trial
Hailey-Hailey disease (HHD) is a debilitating genetic skin disorder, affecting mainly body folds with erythema and painful erosions and blisters. Histopathological findings include epidermal hyperplasia, suprabasilar clefting, dyskeratosis and acantholysis of keratinocytes. A final diagnosis of HHD is usually confirmed based on clinical and histopathological findings in line with genetic testing.
Several treatment options have been proposed for this chronic and disabling disorder, however, there is no reproducibly effective therapeutic for it.
The primary objective is to evaluate the treatment response of guselkumab. Single-center, non-randomized, single-arm, open-label, phase II trial to evaluate the efficacy and safety of guselkumab for the treatment of patients with HHD.
Eligibility criteria
Qualifiers
A documented diagnosis of Hailey-Hailey disease confirmed with clinical, and histopathologic findings
Disease affecting more than one body site with at least moderate severity
If patients are taking other systemic therapies for their HHD (antibiotics, prednisone), they must be taking a stable dose of the other medication(s) for at least 3 months with no plans to change the regimen in the next 6 months. With the exception of antibiotics, methotrexate or low dose prednisone (less than 5 mg daily), use of concomitant immunosuppressants, e.g. azathioprine, etc. and biologics other than TNF-α inhibitors will not be permitted.
Patients must be willing to have skin biopsies, blood collection, and total body photography and to comply with clinic visits
Disqualifiers
Patients with a history of malignancy (except history of successfully treated basal cell or squamous cell carcinoma of the skin over six months prior to first study drug administration).
Patients known to be HIV or hepatitis B or C positive.
Patients planning to receive live vaccines during the duration of the study.
Patients with a positive tuberculin skin test or positive QuantiFERON TB test.
Trial design
Treatments tested in this trial
- Guselkumab
Treatment groups
10 Participants
are divided into 1 treatment groupSponsors and collaborators
Yale University
Lead sponsor
Janssen Scientific Affairs, LLC
Collaborator
Source ClinicalTrials.gov