Study of Diacerein After Maximum Use in Patients With EB

Inclusion Criteria:

- Subject/caregiver is able to comprehend and willing to sign an Informed Consent and/or
Assent Form.

- Subject is male or female at least 12 years of age (Cohort 1) or at least 6 months of
age to 11 years, inclusive (Cohort 2) at screening. US only: subjects must be at least
4 years of age to 11 years, inclusive (Cohort 2) at time of consent.

- The subject must weigh at least 9 kg (19.8 lbs) at Screening.

- Subject has a documented genetic mutation consistent with EB. A blood or saliva sample
will be collected for genetic confirmation if no documented gene mutation data is
available.

- Subject has EB lesions on ≥ 2% body surface area (BSA) and the EB lesions are in the
following body areas:

1. Localized: plantar and/or palmar areas

2. Generalized: arms, legs, torso, hands and feet.

Exclusion Criteria:

- Subject has EB lesions where drug will be applied that are infected (i.e., EB lesions
that require anti-microbial therapy to treat an infection)

- Subject has used any diacerein containing product within 1 month prior to Visit 1

- Subject has used systemic immunotherapy or cytotoxic chemotherapy within 60 days prior
to dosing.

- Subject has used systemic steroidal therapy or has used topical steroidal therapy on
the EB lesions in the application area within 14 days prior to dosing (Note: inhaled,
nasal sprays, and ophthalmic products containing steroids are allowed)

- Subject has participated in an investigational drug trial in which administration of
an investigational study medication occurred within 30 days prior to dosing