Cutis Verticis Gyrata

Excerpt

Cutis verticis gyrata (CVG), also known by the name paquidermia verticis gyrata, cutis verticis plicata, and “bulldog” scalp syndrome, is a rare benign cutaneous disorder that is characterized by convoluted folds and deep furrows of the scalp that mimic cerebral sulci and gyri. It was initially reported in the medical literature in 1837 by Alibert. In 1907, Unna devised the term 'cutis verticis gyrata,' which is the accepted nomenclature ever since. In 1953, Butterworth classified CVG into two forms: primary and secondary. In 1984, the classification system was further broken down into primary essential, primary non-essential, and secondary CVG. Primary non-essential cutis verticis gyrata can be associated with neuropsychiatric and ophthalmological abnormalities. This form now has the name cutis verticis gyrata-intellectual disability (CVG-ID). Secondary and primary non-essential cutis verticis gyrata have associated abnormalities, while primary essential CVG has no associations.