A Neurokinin-1 Receptor Antagonist for the Treatment of Pruritus in Patients With Epidermolysis Bullosa

Inclusion Criteria:

   1. Males or females who are at least 13 years of age.

   2. Willing and able to understand and sign informed assent/consent. Adolescents will need
   a parent or guardian willing and able to give consent.

   3. Clinical diagnosis of epidermolysis bullosa (dystrophic, junctional or simplex).

   4. History of chronic pruritus of at least 6 weeks in duration

   5. On the Screening Visit or Screening phone call, patients must have an NRS pruritus
   score of at least 5 on average itch score in the past 24 hours

   6. Female subjects must be of non-childbearing potential (ie, post-menopausal for at
   least 1 year, had a hysterectomy, or had a tubal ligation) or, if of childbearing
   potential, must have a confirmed negative urine pregnancy test prior to study
   treatment and be willing to use effective contraception for the duration of the trial.
   Effective contraception is defined as follows: oral/implant/injectable/ transdermal
   contraceptives, intrauterine device, condom with spermicide, or diaphragm with
   spermicide. Abstinence or partner's vasectomy is acceptable if the female agrees to
   use effective contraception if she decides to discontinue abstinence or to have sexual
   intercourse with a non-vasectomized partner.

   7. Judged to be in good health based upon the results of a physical examination, medical
   history, and safety laboratory tests.

Exclusion Criteria:

   1. Have any medical condition or disability that would interfere with the assessment of
   safety or efficacy in this trial or would compromise the ability of the subject to
   travel to Stanford or to undergo study procedures or to give informed consent.

   2. Have a history of sensitivity to any components of the study material.

   3. Are females of childbearing potential who are unwilling to use adequate contraception
   or who are breast feeding.

   4. Have any chronic or acute medical condition that, in the opinion of the investigator,
   might interfere with the study results or place the subject at undue risk.

   5. Have chronic renal disease, i.e., serum creatinine greater than 2 times the upper
   limit of normal.

   6. Have chronic liver disease. Subjects with hepatitis B and C who have normal liver
   function may be enrolled.

   7. Have a current malignancy (such as Hodgkin's lymphoma, B or T cell lymphoma, or
   myeloma) or blood cell dyscrasia (e.g., polycythemia or myelofibrosis) that would lead
   to systemic chronic pruritus.

   8. Have a history of thyroid cancer, thyroid nodules, inadequately treated thyroid
   disease, or abnormal TSH or free T4 at screening.

   9. Have a history of abnormalities in adrenal or pituitary function (pituitary adenoma,
   adrenal insufficiency, or adrenal nodule).

10. Screening cortisol level < 3 mcg/dL

11. Unevaluated abnormalities in cortisol, ACTH, or prolactin.

12. Have pruritus of psychogenic etiology (delusions of parasitosis, obsessive compulsive
   disorder and major depression) or neuropathic etiology (due to shingles, spinal cord
   injury or with neurologic deficit).

13. Have pruritus due to urticaria, drug allergy, or infection (such as pityriasis rosacea
   or tinea or active human immunodeficiency virus [HIV]). Note: Subjects with HIV who
   have undetectable viral load, and stable retro-viral therapy may enroll.

14. Have taken investigational medications within 30 days prior to Screening.

15. Are unwilling to discontinue specific medications that, in the view of the
   investigator may have significant interactions with the trial drug, for at least two
   weeks prior to initiation of study and throughout the study period (this includes
   miconazole, delavirdine, conivaptan, Clarithromycin, telithromycin, nefazodone,
   itraconazole, ketoconazole, indinavir, lopinavir, nelfinavir, ritonavir, saquinavir).

16. Are unable or unwilling to maintain their current anti-itch and opioid-based pain
   medications at a stable dosage through the course of the two months of active
   treatment (including but not limited to opioid pain medications, antihistamines, and
   gabapentin)

17. Started or changed medications, creams, or emollients including over-the-counter (OTC)
   preparations or bath oil treatment specifically for relief of pruritus within 30 days
   prior to Screening.

18. Within in the past 12 months, have expressed suicidal ideation with some intent to
   act.

19. Have any social or medical condition (e.g. alcoholism, drug dependency, psychotic
   state) that, in the investigator's opinion, might interfere with the subject's ability
   to comply with the requirements of the protocol.