In 1984, Garden and Robinson (3) improved the classification by proposing new terms: primary essential cutis verticis gyrata for cases in which no other abnormality was found (rare) and primary nonessential, which can be associated with mental deficiency, cerebral palsy, epilepsy, schizophrenia, cranial abnormalities (microcephaly), deafness, ophthalmologic abnormalities (cataract, strabismus, blindness, retinitis pigmentosa), or a combination of these.
Secondary cases of cutis verticis gyrata are associated with the following underlying diseases as mentioned in table below
Type of CVG Associated disease Primary essential CVG None Primary nonessential CVG Neurological: seizures, mental retardation Ocular: cataracts, optic atrophy Secondary * Acromegaly * Amyloidosis * Cylindroma * Connective tissue nevus * Focal mucinosis * Klinefelter syndrome * Leukemia * Myxedema * Melanocyte nevi'hamartomas * Neurofibroma * Nevus lipomatosus * Noonan syndrome * Pachydermoperiostosis * Tuberous sclerosis * Turner syndrome CVG Cutis verticis gyrata In our case a diagnosis of cutis verticis gyrate secondary to pachydermoperiostosis was made.
Treatment of cutis verticis gyrata includes treating the secondary causes, CVG is a cosmetic problem, but psychological repercussions are important.
Based on these clinical findings a diagnosis of cutis verticis gyrata associated with epilepsy was entertained.
Cutis verticis gyrata is a descriptive term for a condition of the scalp in which deep furrows and convolutions are seen that resembles the outer surface of the cerebrum.
Cutis verticis gyrata underrecognized neurocutaneous syndrome.