Introduction
Circumscribed hypertrichosis is usually a developmental defect found in melanocytic naevus and Becker's naevus [1]. A tuft of hair in the lumbosacral region, the so called faun tail naevus is often associated with spina bifida and diastematomyelia [2]. Diastematomyelia is a variety of spinal dysraphism in which there is a cogenital splitting of some part of the spinal cord. Localised hypertrichosis is present in 40% of cases along with subcutaneous lipoma, naevoid skin and dermal sinus [3] or a port wine stain [4].
Case Report
A 7-year old girl child reported to OPD with a tuft of hair over lumbo sacral region since birth. She was otherwise asymptomatic. Family history and pedigree analysis revealed no abnormality.
General examination was normal. Dermatological examination revealed only a tuft of hair overlying the lumbo-sacral region (Fig 1) without any hypo or hypermelanosis. There was no associated dermal sinus or subcutaneous lipoma. Mucosa and nails were normal. Examination of spine and nervous system revealed no motor or sensory impairment.
Fig. 1.
Photograph showing the Faun Tail Naevus in the lumbosacral region
She was diagnosed as a case of Faun Tail Naevus. Radiograph and MRI of lumbo-sacral spine were done for associated spinal dysraphism which was absent. Electroepilation was done in few sittings with satisfactory response.
Discussion
We have reported a rare case of cogenital localised hypertrichosis. Usually they are associated with spinal dysraphism in which there is congenital splitting of some part of spinal cord manifesting clinically either in form of shortening of lower limbs, muscle wasting or weakness, abnormal ipsilateral ankle jerk, impaired sensation of foot or pes cavus and/or claw toes [2]. Numerous radiological abnormalities have been described mainly spina bifida with vertical fusion of lamina. MRI is an excellent investigation giving a particularly clear picture of neural involvement including presence of hydromyelia. Also in 40% cases there is association of localised hypertrichosis. However our case had no clinical or radiological evidence of any neuro-ectodermal involvement other than hair and hence this was a case of isolated faun-tail naevus.
References
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