A Study to Investigate Safety and Efficacy of KB111 for the Treatment of Hailey-Hailey Disease

Purpose

An open-label study to evaluate the safety and efficacy of KB111 in pediatric and adult subjects with Hailey-Hailey Disease

Conditions

  • Hailey Hailey Disease
  • Skin Diseases
  • Lesion Skin

Eligibility

Eligible Ages
Over 12 Years
Eligible Sex
All
Accepts Healthy Volunteers
No

Inclusion Criteria

  • The participant must have read, understood, and signed an Institutional Review Board (IRB)- approved Informed Consent Form and must be willing and able to comply with study procedures and instructions - Aged ≥ 12 years of age at the time of informed consent/assent - Documented clinical diagnosis of HHD with confirmed ATP2C1 variant by genetic testing - A negative pregnancy test prior to initial dose for subjects of child-bearing potential - If taking systemic therapies, subject must be on a stable dosing regimen 1 month prior to Screening and throughout the 12-week treatment period - An acceptable form of contraception must be taken from the first dose (Week 1) through 3 months after the last dose of IP for subjects of childbearing potential

Exclusion Criteria

  • Participation in another clinical study or treatment with an investigational agent 30 days or 5 half-lives, whichever is longer, prior to initial dosing in Week 1 - Participant who is pregnant or nursing - Participant who is unwilling to comply with contraception requirements per protocol (for subjects of child-bearing potential) - Participant who is known to be non-compliant or is unlikely to comply with the requirements of the study protocol, in the opinion of the Investigator - Presence of any dermatologic or systemic condition that might hinder the efficacy of the study treatment or its evaluation, could possibly interfere with the interpretation of study results, or could be judged by the Investigator to be incompatible with the study visit schedule or conduct

Study Design

Phase
Early Phase 1
Study Type
Interventional
Allocation
N/A
Intervention Model
Single Group Assignment
Primary Purpose
Treatment
Masking
None (Open Label)

Arm Groups

ArmDescriptionAssigned Intervention
Experimental
KB111
KB111
  • Biological: KB111
    KB111 is a replication-defective, non-integrating herpes simplex virus (HSV-1)-based vector encoding functional, full-length human ATP2C1 protein combined with a gel for topical application.

Recruiting Locations

Pediatric Skin Research
Miami, Florida 33156
Contact:
Jean Marco Varanese
+1 786-585-5774

More Details

Status
Recruiting
Sponsor
Krystal Biotech, Inc.

Study Contact

Brittani Agostini
412-586-5830
CNE@krystalbio.com

Detailed Description

This clinical study will investigate the safety, tolerability, and preliminary efficacy of open-label KB111 in pediatric and adult subjects with Hailey-Hailey Disease (HHD). Up to 7 subjects will be administered topical KB111 to treat HHD lesions for 12 weeks. KB111 will be administered in the home setting by a trained designee. The Investigator (or designee) will contact subjects weekly (or their parent/legal guardian) to assess for any adverse events or changes in medications, treatments, or procedures. Preliminary efficacy will be assessed by investigator assessments, patient-reported impressions of severity and molecular analysis of skin biopsies. A safety follow-up visit will occur 4 weeks after treatment stops.