|Year : 2015 | Volume
| Issue : 2 | Page : 84-86
Faun tail nevus with neurofibroma: An uncommon cocktail…
Indrashis Podder1, Anupam Das1, Shamick Biswas2, Nilay Kanti Das1
1 Department of Dermatology, Medical College and Hospital, Kolkata, India
2 Department of Radiodiagnosis, IPGMER and SSKM Hospital, Kolkata, India
|Date of Web Publication||9-Apr-2015|
Building -''Prerana'' 19, Phoolbagan, Kolkata - 700 086, West Bengal
Source of Support: None, Conflict of Interest: None
Faun tail nevus is a congenital, posterior midline cutaneous defect, characterized by a circumscribed, hypertrichotic area on the lumbosacral region. It may be a cutaneous marker of underlying spine and spinal cord anomaly. Although, patients with this condition present to us mostly for cosmetic disfigurement, we should be vigilant, and look beyond cosmetic disfigurement, to unearth any underlying spinal anomaly. Here, we present a case of faun tail nevus in a 6-year-old girl, who consulted us merely for cosmetic disability, which was found to be associated with underlying spinal anomalies affecting the lower part of spinal cord and solitary neurofibroma. The clinical importance of such a finding has prompted this report.
Keywords: Anomaly, faun tail nevus, neurofibroma, spinal
|How to cite this article:|
Podder I, Das A, Biswas S, Das NK. Faun tail nevus with neurofibroma: An uncommon cocktail…. Indian J Paediatr Dermatol 2015;16:84-6
|How to cite this URL:|
Podder I, Das A, Biswas S, Das NK. Faun tail nevus with neurofibroma: An uncommon cocktail…. Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Sep 22];16:84-6. Available from: http://www.ijpd.in/text.asp?2015/16/2/84/152129
| Introduction|| |
Faun tail nevus is a rarely reported cutaneous entity, which is characterized by the presence of a hypertrichotic patch affecting the lumbosacral region, since birth. This condition, apart from being a cause of cosmetic embarrassment to the patient, may be associated with underlying spinal dysraphism leading to debilitating symptoms such as back pain or urinary incontinence, thus hampering the patient's quality of life. A proper neurological and radiological evaluation is needed in such cases, to avoid any untoward sequalae of spinal anomaly. We report here a case of faun tail nevus, associated with back pain as a result of underlying spinal diastometamyelia and lumbosacral transitional vertebra (LSTV), with a block vertebra involving the L2, L3 and L4 segments.
| Case report|| |
A 6-year-old girl presented to our outpatient department, with a tuft of hair on her lower back region, since birth. She also complained of occasional low back pain, apart from the obvious cosmetic disfigurement. No history of pain was elicited at the region of abnormal hair growth. Past medical history including developmental milestones was normal. She was born of a nonconsanguineous marriage and has an elder sibling who is not affected. Family history and pedigree analysis were noncontributory. On examination, a tuft of darkly pigmented hair was found on the midline, affecting the lumbosacral region. The tuft of hair formed two tails, one going toward right, and the other in the opposite direction [Figure 1]. On palpation, a nontender, skin colored nodular mass was found at the root of abnormal hair, which was clinically suspected to be a neurofibroma. The parents of the patient refused a skin biopsy. Due to possible spinal anomalies, she underwent a thorough neurological and radiological evaluation. Neurological examination was found to be normal; there being no sensory or motor weakness over her lower extremities, neither was any bladder or bowel incontinence reported. The patient subsequently underwent a 3 Tesla magnetic resonance imaging (MRI) scan of the lumbosacral spine, which revealed Type 1 diastematomyelia with division of the cord into two segments at lower border of L2 vertebra. The two hemicords were separated by an osseo-cartilaginous septum [Figure 2]a]. Sagittal images revealed the presence of a LSTV with a block vertebra involving the L2, L3 and L4 vertebrae [Figure 2]b]. Defective fusion of the posterior elements of the LSTV was also noted with herniation of the thecal sac dorsally while coronal images showed presence of a mild scoliosis with convexity to the right. No evidence of any meningocoele, myelomeningocoele or dorsal dermal sinus was noted. Atrophy of the left-sided erector spinae muscle with diffuse fatty infiltration was also noted on the axial images.
|Figure 2: (a) Axial T2-weighted image (repetition time/echo time, 3820/100 ms) showing the two hemicords (arrowheads) within the thecal sac, separated by a osseo-cartilaginous septum (white arrow), suggestive of Type 1 diastematomyelia. (b) Sagittal T2-weighted image (repetition time/echo time, 2620/110 ms) showing the block vertebra involving L2, L3 and L4 vertebrae (solid arrow) with presence of a lumbosacral transitional vertebra (arrowheads)|
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The patient has been advised regular follow-up and is being currently evaluated for the removal of abnormal hair using long pulsed neodymium-doped yttrium aluminum garnet (Nd: YAG) laser, for better cosmetic results.
| Discussion|| |
Faun tail nevus is a rarely reported congenital cutaneous condition, characterized by the abnormal growth of a tuft of hair, in the midline, affecting the lumbosacral region. This tuft of hair resembles a "faun;" the word referring to an Italian mythological character. Abnormal lumbar hypertrichosis may present in three forms: (1) Simple nevoid hypertrichosis; abnormal hair away from the spine, (2) silky down; soft nonterminal hair, midline, (3) faun tail; wide patch of coarse terminal hair, several inches long, situated at the midline.  In our case, the clinical description matches that of a faun tail.
This condition may be associated with underlying spinal anomalies thus acting as a cutaneous marker. Cutaneous markers may be present in about 76% (43-95%) cases of spinal dysraphism.  We, as dermatologists, should always evaluate such patients thoroughly to unearth any underlying spinal defects at the earliest to avoid the irreversible defect. The broad term, which encompasses all these defects, is called spinal dysraphism, which refers to pathologic conditions resulting from impaired closure of the caudal neuropore.  The most common spinal defects, which have been reported with this condition include spina bifida, intraspinal lipomas, dermal sinuses, lipomeningomyeloceles,  diastematomyelia, ,, tethered cord, , etc., In our case also, MRI revealed Type 1 diastematomyelia along with the presence of a LSTV and a block vertebra involving the L2, L3 and L4 vertebrae. Chander et al. described a case of faun tail nevus associated with aplasia cutis congenital.  while Ustuner et al. reported a co-existence of faun tail nevus with aplasia cutis congenita and fetus papyraceous.  Associated acro-osteolysis, as a result of spinal dysraphism has also been rarely reported.  MRI is an essential diagnostic tool, to rule out these anomalies. Spinal dysraphism was evident in our case also on MRI, as stated before.
Several skin lesions have been described at the base of a faun tail nevus viz., dimple, lipoma, pigmented macule, hemangioma like port wine stain, skin tag, dermoid cyst, neurofibroma etc.  In our case, the nodule at the root of a faun tail was clinically suggestive of solitary neurofibroma. Skin biopsy was proposed to confirm the diagnosis, but was refused by the parents of the patient.
Treatment of this condition essentially consists of removing the excess hair, thus leading to a better cosmetic appearance. Various types of lasers have been found to be useful in this regard viz. Alexandrite laser, Ruby laser, diode, Nd: YAG  and intense pulsed light.  Patra and Ramadasan even showed satisfactory results with electropilation.  However, if clinical features such as severe back pain, urinary incontinence etc., are present, suggestive of grave underlying defects, expert opinion should be sought. In our case, only occasional low back pain was present; so we have planned long-pulsed Nd: YAG epilation.
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[Figure 1], [Figure 2]