IVIg Efficacy Study to Treat Cutaneous Lupus Erythematosus

The ultimate goal of this pilot project is to generate proof-of-concept data showing that
treatment with intravenous immunoglobulin (IVIg) can replace current systemic
immunosuppressive therapy in cutaneous lupus erythematosis (CLE) patients. This project has
relevant clinical implications due to the severe side effects of and lack of response to
current therapies.

From the review of literature, it can be postulated that:

1. the beneficial effects of IVIg for patients with CLE should be prompt, with marked
improvement within a few weeks;

2. clinical improvement should last several weeks after the last infusion; and

3. remission may be prolonged by maintenance IVIg therapy.

Although this is only a non-controlled study, the investigator expects that IVIg will
improve CLE, including those resistant to standard treatments. It is anticipated that
treatment with IVIg will facilitate healing of extensive cutaneous lesions and achieve rapid
remission. Maintenance therapy with repeated monthly pulses of IVIg is expected to keep the
disease in remission during the treatment-free follow up observational period. The results
will provide the basis a multicenter randomized controlled study to identify which CLE
subsets will benefit the most and which protocol will provide the optimal clinical outcome.

Inclusion Criteria:

- Evidence of a personally signed and dated informed consent document indicating that
the subject has been informed of all pertinent aspects of the trial.

- Subjects who are willing and able to comply with scheduled visits, treatment plan,
laboratory tests, and other study procedures.

- Be at least 18 years of age at time of informed consent.

- Have had a diagnosis of CLE

- Currently has active CLE (any subtype) established by standard clinical and histo-
and immunopathologic criteria

- Falls into one of the two following cohorts:

- Cohort 1 - Has received a standard systemic therapy without a therapeutic response
for a minimum of one month

- Cohort 2 - Has not received any systemic treatment

Exclusion Criteria:

- Subject is not over 18 years of age.

- Subject cannot understand or follow directions.

- Subject is a female of child-bearing potential and unwilling to use a form of highly
effective birth control.

- Subject is pregnant, planning to get pregnant, or breast feeding.

- Subject has a known history of immunoglobulin A (IgA) deficiency.