Efficacy of Guselkumab in Treating Hailey Hailey Disease

Description

Several treatment options have been proposed for HHD as chronic and disabling disorder, however, there is no reproducibly effective therapeutic for it.

The primary objective is to evaluate the treatment response of guselkumab. After the participant has been deemed an appropriate candidate for the study and has signed the consent form, they will be enrolled. All participants will be started on guselkumab 100mg at the FDA-approved psoriasis dose and will be seen for clinical follow-up 4, 12, 24 weeks after initiation of therapy. A 12-week follow-up period will be conducted post-administration of the final dose of guselkumab to monitor safety. Laboratory monitoring including Complete Blood Count, Basic metabolic panel, Liver function tests, and pregnancy test (if applicable) will also be performed at 1 month, 3 months, and 6 months after starting therapy. At each visit except Week 20 a full H&P, complete Review of Systems and physical examination will be performed. Outcomes will be assessed and full-body photography will be performed at each visit except Week 20. Research tissue samples (skin biopsies) will be obtained at baseline (prior to therapy) after 24 weeks of treatment in all participants. Research blood will be obtained at screening, baseline and after 4, 12, and 24 weeks of guselkumab therapy.