Partial manifestation of the Proteus syndrome with a striated epidermal nevus.
Furthermore the Proteus-Syndrome is characterized by:
- the hypertrophy of individual structures, such as individual fingers or toes, a whole limb or half of the body
- Tumour-like thickening of the fatty tissue (lipomas) or the connective tissue can also occur.
- Major symptoms of Proteus Syndrome:
- Hemihypertrophia corporis (91% of patients)
- Macrodactyly (88% of patients)
- Subcutaneous mesodermal tumors (76%)
- palmo-plantar cerebriform naevi (65%)
- Exostoses (62%)
- epidermal naevi (50%)
- Scoliosis (50%).