Long Term Open-label Study Evaluating Safety of Diacerein 1% Ointment Topical Formulation in Subjects With Epidermolysis Bullosa Simplex

This is an international, multicenter, open-label, long term extension study evaluating the
safety of topical Diacerein 1% Ointment for the treatment of subjects with EBS. At Visit 1,
EBS subjects who completed the CCP-020-301 double-blind safety and efficacy study or
participated in the CCP-020-101 PK study (feeder studies) who meet all the
inclusion/exclusion criteria will be eligible to complete 2 treatment cycles of CCP-020.

Each treatment cycle will consist of 8 weeks on treatment (once-daily, at home study
medication applications) followed by 8 weeks off treatment (only Investigator approved bland,
non-medicated emollient/moisturizer, routine cleansing products and sunscreens) with a
maximum of 2 treatment cycles allowed for up to 52 weeks.

At Baseline Visit (corresponding to the final study visit of the feeder study), the
investigator will perform a clinical assessment and determine if any of the subject's lesions
require treatment (up to 30% BSA). If the subject has active lesions as determined by the
Investigator's clinical assessment, the subject will initiate a cycle of once-daily, at home
study medication application to their EBS lesions for 8 weeks (treatment period) followed by
an 8-week period where no treatment will be administered. Subjects presenting with no active
lesions (as determined by the Investigator's clinical assessment) will not begin treatment
and instead, will be instructed to return to the clinic for re-evaluation in 8 weeks or upon
worsening of EBS lesions, whichever happens first.

Inclusion Criteria:

- In the opinion of the Investigator, the subject is capable of understanding and
complying with protocol requirements.

- The subject or, when applicable, the subject's legally acceptable representative signs
and dates a written, informed consent/assent form and any required privacy
authorization prior to the initiation of any study procedures.

- Subject has a documented genetic mutation consistent with EBS.

- Subject has completed study CCP-020-301 or participated in study CCP-020-101.

- Subject/caregiver agrees to report use of any topical therapies applied to EBS lesions
(e.g. medicated cleansers, bleach cleansers, bleach baths, topical antiseptics,
topical disinfectants, etc.).

- If the subject is a woman of childbearing potential, she has a negative urine
pregnancy test and agrees to use an approved effective method of birth control, as
defined by this protocol, for the duration of the study.

- Subject is non-lactating and is not planning for pregnancy during the study period.

- Subject is willing and able to follow all study instructions and to attend all study
visits.

Exclusion Criteria:

- Subject has EBS lesions to be treated that are infected (i.e., EBS lesions that
require topical antibiotic therapy to treat an infection).

- Subject has evidence of a systemic infection or has used systemic antibiotics within 7
days prior to Baseline Visit.

- The subject was discontinued from the feeder study due to an adverse event judged to
be related or possibly related to the study medication.

- Subject has experienced a change in clinical status from the feeder study that, in the
investigator's opinion, puts the subject at undue risk to participate.