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Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 135-137

Diffuse wooly hair mimicking as uncombable hair syndrome!

Department of Dermatology, Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka, India

Date of Web Publication27-Mar-2017

Correspondence Address:
Samipa Samir Mukherjee
Department of Dermatology, Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.203001

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How to cite this article:
Mukherjee SS, Chandrashekar B S. Diffuse wooly hair mimicking as uncombable hair syndrome!. Indian J Paediatr Dermatol 2017;18:135-7

How to cite this URL:
Mukherjee SS, Chandrashekar B S. Diffuse wooly hair mimicking as uncombable hair syndrome!. Indian J Paediatr Dermatol [serial online] 2017 [cited 2021 Jun 23];18:135-7. Available from: https://www.ijpd.in/text.asp?2017/18/2/135/203001


Woolly hair is a rare congenital abnormality of the structure of the scalp hair characterized by extreme kinkiness of hair and tightly coiled curls involving part or the entire scalp occurring in an individual of nonnegroid origin as first described by Gossage in 1907.[1] It may present as isolated finding or can have underlying syndromic associations. Diagnosis of wooly hair mainly is clinical with corroborative evidence through light microscopy showing the inability to focus under the microscope. The clinician may face a diagnostic challenge in differentiating from uncombable hair syndrome in certain cases owing to the similarity of clinical presentation with reference to the texture, lustre and manageability. We herein present a case of isolated diffuse wooly hair mimicking uncombable hair syndrome and suggest the importance of differentiating between the two conditions prognostically.

A 12-year-old female child, born of a normal delivery to a nonconsanguineous marriage presented with complaints of difficult to manage hair since a few months after birth. There was a typical history of the hair standing on end and not flattening out on combing. There was a history of repeated traction combing of the hair and tight tying up of hair, in an effort to flatten the hair by the mother and child. On clinical examination, her hair was lustreless, appeared frizzy, and stuck out from the patient's head over the temporoparietal area with a regular wave pattern. The hair over the occipital and nuchal area was however tightly coiled in curls unlike the temporoparietal area [Figure 1]. Hair clippings and hair pull test were negative. Light microscopic examination of the hair shaft revealed an inability to focus the strand in one field. Electron microscopy of the cut section could not be performed due to unavailability. General physical examination was unremarkable, and there were no associated abnormalities of the skin, nails, or teeth. She had refractory errors for which she was treated. The family history was negative for any dermatologic conditions and was otherwise noncontributory.
Figure 1: Hair that cannot be flattened, tight curls noted over the occipital area, regular waves affecting the temporoparietal area

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Based on the history, clinical evaluation, and microscopic evaluation, a diagnosis of isolated diffuse wooly hair mimicking uncombable hair syndrome due to repeated traction was made.

Uncombable hair syndrome is a distinctive hair shaft defect characterized by disorderly hair that stands out from the scalp and cannot be combed flat. The underlying structural hair shaft anomaly is longitudinal grooving, which appears triangular or kidney-shaped in cross section.[2] Unruliness of hair caused by external factors may mimic this condition and thereby pose a clinical challenge in the diagnosis of the condition. Friction, photodamage, hair treatments, exposure to ultraviolet radiation damages hair fibers, wind and sunlight can lead to dryness, rough surface texture, decreased color and lustre, withering, and increased stiffness and brittleness.[3],[4] Although electron microscopy forms the gold standard to differentiate between wooly hair, uncombable hair and acquired progressive kinking of hair accessibility limits its usage in day-to-day practice. As seen in our case, the presence of tightly coiled curls in the occipital and nuchal area clinched the diagnosis of diffuse wooly hair which was overshadowed by the presence of unruly hair over the parietotemporal area. The presence of unruly/uncombable hair over the parietotemporal area can be explained by the history of repeated traction to the wooly hair leading to loosening of the curls and giving the picture of wavy, frizzy strands that cannot be flattened.

Clinical examination of the full scalp with a good history most of the times clinches diagnosis. The importance of differentiating between the two conditions is beneficial for prognosis and to rule out underlying associations. Uncombable hair generally spontaneously resolves around puberty which is not the case with wooly hair. The finding of uncombable hair is usually an isolated finding and as such it is not associated with physical, neurologic, or mental abnormalities, whereas wooly hair may be an isolated finding or be a part of the systemic disease having a syndromic association like that of Carvajal and Naxos.[5]

We herein report the case to highlight the importance of complete clinical examination which helps to arrive at a diagnosis and also the fact that mimickers must be kept in mind while dealing with genotrichoses conditions.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Gossage AM. The inheritance of certain human abnormalities. Q J Med 1907;1:331-47.  Back to cited text no. 1
Hebert AA, Charrow J, Esterly NB, Fretzin DF. Uncombable hair (pili trianguli et canaliculi): Evidence for dominant inheritance with complete penetrance based on scanning electron microscopy. Am J Med Genet 1987;28:185-93.  Back to cited text no. 2
Jeon SY, Pi LQ, Lee WS. Comparison of hair shaft damage after UVA and UVB irradiation. J Cosmet Sci 2008;59:151-6.  Back to cited text no. 3
Santos Nogueira AC, Joekes I. Hair color changes and protein damage caused by ultraviolet radiation. J Photochem Photobiol B 2004;74:109-17.  Back to cited text no. 4
Vasudevan B, Verma R, Pragasam V, Badad A. A rare case of woolly hair with unusual associations. Indian Dermatol Online J 2013;4:222-4.  Back to cited text no. 5
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