PRIMARY NON-ESSENTIAL CUTIS VERTICIS GYRATA: REPORT OF A CASE.
Cutis verticis gyrata (CVG) is a rare transformation of the scalp characterized by ridges and furrows resembling surface of the brain. It has primary and secondary types. Patients with primary CVG have normal skin and does not have an underlying pathological process. It is further subdivided into non-essential CVG and essential CVG based on the presence or absence of ophthalmological abnormalities or neuropsychiatric problems such as mental retardation, cerebral palsy, schizophrenia or epilepsy. The secondary CVG is always a manifestation of an underlying inflammatory, neoplastic or a systemic disorder. We report here a case of cerebral palsy in a 16-year-old boy who presented with fourteen months' history of progressive deformity of head and face and was diagnosed as primary non-essential CVG.
Key Words: Cutis verticis gyrata, Cerebral palsy, Non-essential cutis verticis gyrata.
Cutis verticis gyrata (CVG) is a unique condition of scalp deformation characterized by folding and furrowing of scalp in a pattern that resembles the crests and gyri of cerebral cortex. It is more common in males compared to females with an approximate prevalence of 1 in 100,000 in males and 0.026 in 100,000 in females 1. It has primary and secondary types. Patients with primary CVG have normal skin and is further subdivided into non-essential CVG and essential CVGs based on the presence or absence of neuropsychiatric problems such as mental retardation, cerebral palsy (CP), schizophrenia or epilepsy 2. In secondary CVG, the scalp has pathologic changes secondary to either local inflammation, neoplasia or a systemic illness. We present here a case who presented with symptoms of progressive head and face deformity and was found to have primary non-essential CVG.
We present here case of a 16-year-old boy from rural Punjab who presented with fourteen months' history of progressive deformity of head and face. According to the parents, the patient had mild weakness in right arm and leg since infancy but had improved a lot after taking some herbal medicines. The patient had no history of fits, visual problems, mental retardation or a psychiatric disorder. There was no history of recent drug in-take. The family history was positive as the elder brother had similar head and facial deformities.
On examination, he had wasting of right thenar muscles (Figure 1A). Right foot was slightly inverted on free hanging (Figure 1B). A prominent abdominal hernia and a groove over lumbar spine was evident (Figure 1C and 1D). He had a normal power except for right interossei and abductor pollicis brevis that had a power of-4/5. The deep tendon reflexes were normal, however, the Babinski sign was positive on the right side. He had mild acne over upper chest and back (Figure 2A and 2B). On scalp examination, he had multiple horizontal non-reducible furrows over the scalp (Figure 2C and 2D). There were no skin lesions over the scalp. The child was asked for a head shave to have a clearer scalp view but he refused. His IQ evaluation revealed border line deficiency.
His complete blood count, renal, thyroid and liver function tests were normal. The serum growth hormone levels were within the normal range. The X-ray skull did not show any abnormality. The computerized tomography (CT) scan was normal except for scalp convolutions. The nerve conduction studies for right arm and leg were normal except for moderate median nerve compression at right wrist. The scalp skin biopsy was refused by the patient and his parents. Thus, the patient was diagnosed as a case of primary non-essential CVG. The weakness in right arm and foot inversion were probably sequalae of right sided hemiplegic CP due to which he was assumed to be suffering from non-essential type of primary CVG. The patient was suggested surgical resection of the lesions that his parents refused because of financial problems.
Primary CVG is a rare condition, unique in appearance due to progressive folding and furrowing of the scalp simulating the cortical convolutions. The usual age of onset of primary CVG is after puberty and before 30 years of age 2. Essential primary CVG is not associated with other co morbid conditions and is more prevalent in men3. Most of the cases are sporadic, but autosomal recessive and autosomal dominant inheritance have also been seen 4,5. On the other hand, the non-essential form occurs in association with various neurological conditions like microcephaly, mental retardation and epilepsy 3 or ophthalmological changes such as cataract, strabismus or blindness 3. Secondary CVG manifests with an underlying pathology and may exist since birth 2.
The pathology may be the result of a local inflammatory (eczema, psoriasis, folliculitis, impetigo, erysipelas, atopic dermatitis and acne conglobata) 4,6,7 or a neoplastic process or systemic illness (leukemia, neurofibroma, myxedema 2,4,7. Secondary CVG has also been reported following treatments with vemurafenib and whole-brain radiotherapy 8. Establishing the appropriate diagnosis is paramount in management. Diagnosis of primary CVG is clinical and does not require laboratory tests. In secondary cases, the presentation and the associated disease determine the laboratory tests need to be done. Magnetic resonance imaging and CT scan are essential in CVG presenting at birth or when associated with neurologic and/or ophthalmologic abnormalities in ruling out concomitant structural abnormalities of brain 9.
It is recommended to obtain a histopathological analysis of the affected area; the picture is normal in primary type whereas in the secondary form it depends on the un-derlying disease 5. There is only single report of CVG previously from Pakistan. It was a 60-year-old lady with skin lesions due to the effects of infiltrating intraductal breast carcinoma 2. Our case was the first report of primary non-essential CVG from Pakistan who had associated effects of CP.
Primary CVG carries a better prognosis, although surgical intervention is required to halt the disease progression and further follow-ups are mandatory 10. In secondary cases, the nature of underlying process determines the treatment. Management in primary essential CVG involves maintenance of skin hygiene and cosmesis along with psychological interventions 11-14. Surgical resection of the lesions is often requested for psychological or esthetic reasons. Choices of treatment in CVG depend on size of the lesion, the underlying cause and the patient's requests or preferences. Surgical treatment options include primary repair, serial excision and skin grafting and local flap and serial tissue expansion. Patients who refuse to undergo any surgical intervention need 3-6 months' follow up to assess progression of the lesions. There are no specific medicines available for the treatment of CVG.
Primary nonessential CVG is an extremely rare condition. The diagnosis can only be made after excluding secondary associations through appropriate physical evaluation and investigations. The mainstay of treatment is surgery that largely depends on the patient's choice.
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|Publication:||Journal of Postgraduate Medical Institute|
|Date:||Jun 30, 2018|
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