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Year : 2012  |  Volume : 4  |  Issue : 1  |  Page : 42-43  

Woolly hair nevus: A rare entity

Department of Dermatology, SRM Medical College Hospital and Research Centre, SRM Nagar, Potheri, Tamil Nadu, India

Date of Web Publication12-May-2012

Correspondence Address:
V Venugopal
New No-22, Dr. Nair Road, B-4, Vaishali Apartments, T-Nagar, Chennai - 600 017, Tamilnadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-7753.96090

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Woolly hair nevus is a rare non-hereditary focal condition characterized by unruly and tightly coiled hair. It can appear in childhood or adolescence and may be associated with epidermal or melanocytic nevus. Patients presenting with woolly hair must be examined completely to rule out cardiofaciocutaneous and Noonan syndrome.

Keywords: Woolly hair nevus,woolly hair, kinky hair

How to cite this article:
Venugopal V, Karthikeyan S, Gnanaraj P, Narasimhan M. Woolly hair nevus: A rare entity. Int J Trichol 2012;4:42-3

How to cite this URL:
Venugopal V, Karthikeyan S, Gnanaraj P, Narasimhan M. Woolly hair nevus: A rare entity. Int J Trichol [serial online] 2012 [cited 2023 Feb 9];4:42-3. Available from: https://www.ijtrichology.com/text.asp?2012/4/1/42/96090

   Introduction Top

Woolly hair nevus is a rare condition, characterized by unruly and tightly coiled hair, which is not genetically determined. It may be localized to one or several areas of the scalp. We report a case of woolly hair nevus in a child.

   Case Report Top

A 5-year-old girl child presented with abnormal patch of hair since 2 years of age. Her parents noticed a single patch of curling and coiling of hair along with altered texture over the left side of scalp. Her parents felt the patch to be unruly and unsightly and hence attempted repeated tonsuring. In spite of this, the patch of hair remained the same. Birth and development history of the child was normal. Her family members did not have similar complaints. On examination, we noticed a solitary circumscribed patch of size 6×4 cm located over the left frontoparietal region of scalp. The hair over the patch had an altered texture, was lighter in color, thinner, tightly coiled, and curled giving an unkempt appearance [Figure 1]. The skin over the patch was apparently normal. Examination of rest of the scalp, teeth and nails were also normal. There was no evidence of palmoplantar keratoderma and evidence of any epidermal nevus elsewhere. Ophthalmic and cardiac evaluation did not reveal any abnormality. There was no evidence of precocious puberty. Based on the above findings, we arrived at a diagnosis of localized woolly hair nevus. Hair microscopic examination showed non-specific features. Scalp biopsy revealed normal hair follicles and appendages [Figure 2]. Routine hemogram, serum biochemistry, electrocardiogram and echocardiogram were normal.
Figure 1: A well-circumscribed patch of tightly coiled woolly hair

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Figure 2: HPE of scalp showing normal hair follicle and appendages

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   Discussion Top

Woolly hair is characterized by tightly coiled hair occurring over the entire scalp or part of it in an individual of non-African origin. Hutchinson et al. have classified woolly hair types as follows: 1. Hereditary woolly hair, 2. Familial woolly hair, 3. Symmetrical circumscribed allotrichia 4. Woolly hair nevus. [1]

The term woolly hair nevus was first described and coined by Dr. Fred Wise in 1927. It is a nevoid condition characterized by a circumscribed patch of unruly and curled hair with an altered texture. Both sexes are equally affected. [2] At birth, the hair may be normal as in our child. It becomes coarser with age giving an unsightly appearance. This condition may be associated with melanocytic or epidermal nevi [3] , mongolian spots [4] elsewhere on the skin. Ocular defects like persistent pupillary membrane, [2],[5] retinal defects, [6] and precocious puberty have also been associated. [7] Electron microscopic findings include twisting of hair shaft and abnormal cuticle formation. [8]

Woolly hair nevus is not associated with cardiac abnormalities as in Carvajal or Naxos disease in which desmoplakin and plakoglobin mutations are known to occur, respectively. [9] Our patient presented with an isolated woolly hair nevus without any associations, which has been reported very rarely. However, this patient must be followed up regularly to detect any cardiac defects which may present later. [10]

   References Top

1.Hutchinson PE, Cairns RJ, Wills RS. Woolly hair. Clinical and general aspects. Trans St John Hosp Dermatol Soc 1974;60:160-76.  Back to cited text no. 1
2.Stieler W, Otte HG, Stadler R. Multiple woolly hair naevi with linear epidermal nevus and persistant pupillary membrane. Hautarzt 1992;43:441-5.  Back to cited text no. 2
3.Manzoor S, Mir NA, Qayoom S. Verrucous epidermal naevus associated with woolly hair naevus. Indian J Dermatol Venereol Leprol 2003;69:37-8.  Back to cited text no. 3
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4.Usha V, Nair T. Woolly hair nevus - A case report. Indian J Dermatol Venereol Leprol 1997;63:330-1.  Back to cited text no. 4
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5.Phiske MM, Saualka SS, Ovhal AG, Jerajani HR. Woolly hair nevus arising on a verrucous epidermal nevus with contralateral persistant pupillary membrane. Eur J Pediatr Dermatol 2006;16:13-6.  Back to cited text no. 5
6.Taylor AE. Hereditary woolly hair with ocular involvement. Br J Dermatol 1990;123:523-5.  Back to cited text no. 6
7.Tay YK, Weston WL, Ganong CA, Klingensmith GJ. Epidermal naevus syndrome: Association with central precocious puberty and woolly hair naevus. J Am Acad Dermatol 1996;35:839-42.  Back to cited text no. 7
8.Goldin HM, Bronson DM, Fretzin DF. Woolly hair nevus - A case report and study by scanning electron microscopy. Pediatr Dermatol 1984;2;41-4.  Back to cited text no. 8
9.Yesudian PD. Hair abnormalities in genetic disorders of junctions. Int J Trichology 2009;1:15-7.  Back to cited text no. 9
10.de Oliveira JR, Mazocco VT, de Arruda LH. Woolly hair nevus syndrome. An. Bras. Dermatol. vol.79 no.1 Rio de Janeiro Jan./Feb. 2004. Available from http://dx.doi.org/10.1590/S0365-05962004000100013 [Last cited on 2011 Dec 12]  Back to cited text no. 10


  [Figure 1], [Figure 2]

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