Basal cell nevus syndrome is a rare
autosomal dominant disorder with a prevalence
of between 1in 60,000 to 1in 120,000. This disorder is
associated with a panoply of phenotypic
that includes developmental
anomalies and tumors particularly basal cell
carcinoma. The genetic abnormality in almost all
known cases is a mutation in the PATCHED] gene
which is essential for normal body and limb
patterning.?
abnormalities
We report a 50-year-old Filipina who
suffered from multiple recurrent pigmented
papules, plagues, nodules, and tumors on the face
with the first tumor appearing at age 20.
Basal Cell Nevus Syndrome