Blue vs Red Light During Levulan Based Photodynamic Therapy in Patients With Basal Cell Nevus Syndrome

This is a pilot intra-patient comparative study to evaluate the ability of cyclic
Photodynamic Therapy (PDT) using Red (635 nm) or Blue (400 nm) light with 5-Aminolevulinic
acid, to eliminate BCC skin cancers that occur in patients with Basal Cell Nevus Syndrome
(Gorlin-Goltz Syndrome).

Patients will receive 3 cycles of Red light and Blue light PDT treatments, for a total of 6
treatments, over a 4 month period. Cycles will be spaced 2 months apart. Each cycle consists
of a double course of PDT treatment with treatments spaced one week apart. Every subject
will be treated with two light sources, blue and red, according to randomized assignments
made to left side or right side of the body. There will be a final assessment visit at month
6.

Inclusion Criteria:

- Men, women, and children of any age or ethnic group who meet eligibility

- Patients under 18 years of age must be accompanied and co-consented, by a parent or
legal guardian.

- Patients must have a diagnosis of Basal Cell Nevus Syndrome (BCNS)

- For diagnosis of BCNS, the patient must have either 2 major criteria, one major and
two minor criteria, or one major criterion plus molecular confirmation of a PTCH1
gene mutation

- Major criteria are:

- (1) BCC prior to age 20 years, or excessive number of BCCs out of proportion to
prior sun exposure and skin type

- (2) keratocyst of the jaw prior to age 20

- (3) palmar or plantar pitting

- (4) lamellar calcification of the falx cerebri

- (5) medulloblastoma

- (6) first degree relative with BCNS

- Minor criteria are:

- (1) rib anomalies, or other specific skeletal malformations including
kyphoscoliosis and short 4th metacarpals

- (2) macrocephaly

- (3) cleft/lip or palate;

- (4) fibroma of the heart or ovary

- (5) ocular abnormalities

- (6) other rare abnormalities

- At least two BCC tumors, preferably more, located in different body regions or
located greater than 10 cm apart in locations that can be reproducibly separated into
red and blue illumination fields

- Female subjects are not pregnant or nursing or planning to become pregnant during the
study

- Subjects must have the ability to understand and the willingness to sign a written
informed consent document

Exclusion Criteria:

- Pregnant, planning on getting pregnant or nursing

- Currently participating in another clinical trial

- Using any topical treatment for their BCC tumors, unless discontinued at least 1
month prior

- Currently being treated for other cancers with medical or radiation therapy

- Patients with a known hypersensitivity to 5-aminolevulinic acid or any component of
the study material

- Patients with a history of a photosensitivity disease, including porphyria cutanea
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