Secondary Cutis verticis gyrata in a Patient With Tuberous Sclerosis?

Dear Editor,

We have read with interest the article published by Duménigo Arias et al. about a male patient with a giant cerebriform nevus of the scalp,¹ and we would like to made two brief comments.

Cutis verticis gyrata (CVG) is characterized by the presence of circumvolutions and deep grooves that imitate the cerebral surface; it is more frequent in men and is classified into primary or secondary types.² The secondary type is associated with systemic diseases such as acromegaly and tuberous sclerosis, inflammatory dermatosis such as psoriasis and eczema, and numerous skin tumors like plexiform neurofibroma, hamartoma, cylindroma, dermatofibroma, collagenoma, nevus lipomatosus, sebaceous nevus and congenital melanocytic nevus.³

Cerebriform intradermal nevus predominates in females and constitutes up to 12.5% of all CVG. Normally, it is a congenital, non-hyperpigmented, alopecic lesion located in the parietal–occipital region that grows rapidly between the ages of 5 and 10.⁴ Part of its importance lies in the potential risk for developing melanoma (4.5%).⁵ We believe that the cerebriform clinical morphology and the case history presented are compatible with the diagnosis of nevus, but a pathology study is necessary to exclude other causes of CVG, such as hamartomas or neurofibromas.

Furthermore, in one of the images of the patient provided by the authors, several skin lesions are observed on the forehead as well as multiple lesions on the nose and nasolabial folds, suggestive of fibrous plaque and angiofibromas, respectively. These are typical manifestations of tuberous sclerosis complex (TSC), which is a cause of CVG and is related with tumors like collagenomas, another possible cause.^4,6,7 It should also be noted that there has been a recent report of a giant congenital nevus of the scalp in a girl with TSC.⁸

In conclusion, we feel it is important to complete the clinical history and the physical examination of the patient with a histopathology study of a cerebriform scalp tumor. This would allow us to know whether the patient has TSC and confirm the nature of the lesion originating the secondary CVG, and thus increase the value of this interesting case.

Conflict of Interest

We have received no grant support, nor have we made previous presentations at congresses. Both authors have contributed to the authorship of this manuscript.