A rare cutis verticis gyrata secondary to cerebriform intradermal nevus: case report and literature review

Abstract

Background Cutis verticis gyrate (CVG) is a rare morphologic syndrome that presents with hypertrophy and folding of the scalp. CVG can be classified into three forms: primary essential, primary non-essential, and secondary. Cerebriform intradermal nevus (CIN) is a rare cause of secondary CVG. We are here to report a rare case of CVG with an underlying CIN and discuss the clinical course, treatment options, and critical screening guidelines for these patients. Case presentation A 25 year-old male patient presented with a chief complaint of generalized hair loss in the scalp parietaloccipital region for a duration of 1 year and the hair loss area was occasionally accompanied by mild itching. The hair loss started gradually and worsened over time. In addition, he had scalp skin folds resembling the ridge and furrow of the cerebral cortex in the parietaloccipital region since birth. Physical examination revealed hypertrophy and formation of folds in the parietal-occipital area, forming 5 to 6 furrows and ridges. The size of the cerebriform mass was about 12.0 cm × 8.5 cm, without other skin lesions. Diffuse non-scarring hair loss was distributed on the posterior-parietal scalp, mid-parietal scalp and superior-occipital scalp. The diseased tissue of the patient's parietaloccipital area was excised under general anesthesia. The postoperative pathological examination of the tissue excised showed that there were dense intradermal melanocytic nevus, so the patient was diagnosed with secondary CVG caused by CIN. At the 2 year follow-up, there were no obvious changes in the lesions. Conclusions CIN must be differentiated from other conditions that manifest as CVG, including primary essential or non-essential CVG and secondary CVG caused by other reasons. Each CIN patient requires a specific decision of whether to excise the lesion surgically or follow a wait-and-see policy, depending on the patient's will and specific condition. Surgical treatment may be performed when there is an aesthetic demand. However, clinical observation and close follow-up is also a good treatment choice for patients with stable disease or mild symptoms.

Cerebriform intradermal nevus manifesting as cutis verticis gyrata

“…[6] e average number of skin folds with CVG in one study was six, though it may vary from two to as many as twenty folds. [7] e direction of the folds is usually anterior to posterior in vertex but may be transverse over the occipital region or sometime can be irregular. In primary CVG, folds are usually symmetrical and are asymmetrical in secondary form.…”

Cutis Verticis Gyrata and Quality of Life: Clinical Report of 13 Cases

“…To the best of our knowledge, this is the first study to report CVG secondary to neurofibroma, folliculitis fibrosis, dissecting cellulitis, and lipoedema. Previous studies reported CVG secondary to acromegaly (14,15) , cerebriform intradermal nevus (2,3) . We were unable to find the primary non-essential form of CVG, which is rare and exceptional.…”

“…The primary essential CVG is not linked to any other diseases, whereas the primary nonessential form is linked to mental deficiency, neurological diseases, and ophthalmological disorders (2) . Secondary CVG can be caused by many factors, including inflammatory dermatoses, systemic diseases, infiltrates, and cutaneous neoplasms (3) .…”

“…Clinical, pathological, and radiological features of cutis verticis gyrata patients were reported in many studies (2)(3)(4)(5)(6) . As far as we know, patients' quality of life with CVG has not been previously investigated in the literature.…”

Primary Essential Cutis Verticis Gyrata: A Case Report

“…Dear Editor, Cutis verticis gyrata (CVG) is a rare scalp condition characterized by excessive subcutaneous tissue proliferation, resulting in folds resembling cerebral gyri. 1 First docu-mented by Jean-Louis-Marc Alibert in 1837 and later named by Paul Gerson Unna in 1907, it is also known by various names like Robert-Unna syndrome, bulldog scalp, and corrugated skin. The estimated prevalence rate in adults is 1 in 100 000 for men and 0.026 in 100 000 for women.…”