Inclusion Criteria:

- A positive genetic identification of the N171K mutation in the keratin 6a gene from a
CLIA certified laboratory;

- Witnessed, signed informed consent approved by Institutional Review Board/Ethics
Committee;

- A signed Health Information Portability and Accountability Act (HIPAA) authorization
form which permits the use and disclosure of patient's individually identifiable
health information for those enrolled in the United States of America;

- Male or female subjects of any race 10 years of age and older;

- Complete physical examination and medical history indicating no abnormalities that
will interfere with study objectives;

- Normal or not clinically significant baseline laboratory tests, including hemogram,
ANA, serum chemistry panel, urinalysis, C3a, Bb, and APTT,PT;

- Negative pregnancy test (females only).

Exclusion Criteria:

- Females of childbearing potential not using a highly effective method of birth
control (e.g. implants, injectables, combined oral contraceptives, some intrauterine
contraceptive devices) during the study;

- Diabetes mellitus requiring treatment other than diet and exercise;

- Treatment of any type for cancer within the last six months;

- History of any significant internal disease;

- Subjects who are known to be allergic to any of the test product(s) or any components
in the test product(s) or history of hypersensitivity or allergic reactions to any of
the study preparations;

- History of street drug or alcohol abuse;

- Any patient not able to meet the study attendance requirements;

- Subjects who have participated in any other trial of an investigational drug or
device within 60 days prior to enrollment or participation in a research study
concurrent with this study.