The
Proteus syndrome is a recently described
congenital harmatosis consisting of numerous clinical features of great variety. Mainly affected are the
musculoskeletal system, primarily by hemihypertrophy, macrodactyly,
exostoses and kyphoscoliosis, and the
skin and the
subcutaneous tissue, primarily by
pigmented nevi and subcutaneous
tumors. These findings are diagnostic features of
Proteus syndrome. We
report typical manifestations of
Proteus syndrome in a 12-year-old boy with brief
review of
literature.