Ixekizumab in the Treatment of Bullous Pemphigoid

Subjects eligible for inclusion in this study have to fulfill all of the following Inclusion criteria:

• Subjects must be able to understand and comply with the requirements of the study and communicate with the investigator. Subjects must give written, signed, and dated informed consent before any study related activity is performed. When appropriate, a legal representative will sign the informed consent according to local laws and regulation
• Both men and women must be at least 18 years of age at the time of screening
• Subjects must have clinical, histological, and serological features of BP
• Urticarial plaques and/or vesicles and bullae
• Characteristic eosinophilic spongiosis and/or subepidermal separation of the skin
• Positive direct immunofluorescence (IgG and or C3 at the basement membrane zone) or indirect immunofluorescence (IgG on the roof of salt- split skin) or positive serologies on ELISA for BPAG1 or BPAG2
• Subjects must have treatment naive BP or treatment refractory disease, as defined by failure of at least one established treatment for BP
• Candidate for systemic therapy, defined by
• involvement of greater than 5 percent body surface area or moderate to extensive disease as defined by: the mean number of new bullae and urticarial plaques that have appeared over the course of 3 days as determined by the investigator or referring physician (moderate disease defined by greater than 1 and less than 10 new bullae and greater than 5 urticarial plaques and extensive disease by greater than 10 new bullae)
• Failure of prior therapy
• Topical treatment
• Systemic immunosuppressant
• Oral antibiotics and/or niacinamide

Exclusion Criteria

Subjects fulfilling any of the following criteria are not eligible for inclusion in this study. In order to ensure the recruitment of a representative sample of all eligible subjects, the investigator may apply no additional exclusions.

• Forms of BP other than classic BP (e.g. mucous membrane BP, Brunsting-Perry BP, p200 BP, p105 BP, or BP with concomitant pemphigus vulgaris Drug-induced BP (e.g., new onset or current exacerbation from angiotensin converting enzyme inhibitors, penicillamine, furosemide, phenacetin)
• Subjects who are receiving treatments known to worsen BP and use of penicillamine or phenacetin and those on angiotensin converting enzyme inhibitors or furosemide who have not been on a stable dose at least 4 weeks prior to enrollment.
• Ongoing use of prohibited treatments.
• Previous exposure to Ixekizumab or any other biologic drug directly targeting IL-17A or IL-17 (receptor A)RA
• Use of any other investigational drugs within 5 half-lives of the investigational treatment before study drug initiation or until the pharmacodynamics effect has returned to baseline, whichever is longer
• Previous use of IL-20 monoclonal antibody
• Pregnant or nursing (lactating) women (pregnancy is defined as the state of a female after conception and until the termination of gestation, confirmed by a positive human chorionic gonadotropin (hCG) laboratory test)
• Women of childbearing potential [Post-menopausal or not of child-bearing potential is defined by: 1 year of natural (spontaneous) amenorrhea or Surgical bilateral oophorectomy (with or without hysterectomy), total hysterectomy or tubal ligation at least 6 weeks ago. Oophorectomy alone must confirmed by follow up hormone level assessment to be considered not of child-bearing potential, defined as all women physiologically capable of becoming pregnant, unless they are using basic methods of contraception which includes:
• Total abstinence (Periodic abstinence and withdrawal are not acceptable methods of contraception)
• Female sterilization (bilateral oophorectomy with or without hysterectomy), total hysterectomy, or tubal ligation at least 6 weeks before taking study treatment. Oophorectomy alone requires follow up hormone level assessment for fertility.
• Male sterilization (at least 6 months prior to screening). The