A female infant, born of non-consanguineous marriage, presented with edema of hands and feet and loose folds of skin on the vault of skull which her parents noticed from birth. The baby was a term normal delivery. Her birth and developmental history was otherwise uneventful. On head to foot examination, the baby presented with 6-8 loose puckered folds of skin, extending from anterior to posterior in the saggital plane, spanning the vertex and occipital region. The folds were spongy, non tender with overlying hair being sparse. Besides, she had loose folds of skin at the nape of her neck and non-pitting edema of her hands and feet. G-Band karyotype revealed 45 XO. Cutis Verticis Gyrata (CVG) is a rare entity with an estimated prevalence of 1 in 100,000 with an estimated male to female ratio of 6:1. CVG exists in primary and secondary forms. Primary forms exist in primary essential (in which no other abnormality is found) and primary non essential (that can be associated with cerebral palsy, epilepsy, and cranial or ophthalmologic abnormality. Secondary forms are associated with pachydermatosis, melanocytic nevi, dermatofibroma and some syndromic associations like Noonan syndrome, Turner syndrome and many more. In our case, the baby had Turner syndrome, as evident by the karyotyping report. Hence this was a secondary cause of CVG. The close differentials are acromegaly, cutis laxa (elastolysis), cylindroma, and pachydermo-periostosis. The course of the disease, in majority of cases is benign except in primary cases caused due to cerebriform melanocytic naevus, close follow-up is required to detect early malignant transformation. Treatment includes maintenance of optimum hygiene of the skin folds and surgical correction of these folds by laser.

Fig.1 Cutis Verticis Gyrata.