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CASE REPORT
Year : 2020  |  Volume : 21  |  Issue : 2  |  Page : 135-137

Anterior cervical hypertrichosis with linear and whorled nevoid hypermelanosis in a 12-year-old girl: A rare association


Department of Skin and VD, GMC, Kota, Rajasthan, India

Date of Submission30-Nov-2019
Date of Decision24-Dec-2019
Date of Acceptance30-Jan-2020
Date of Web Publication01-Apr-2020

Correspondence Address:
Devendra Yadav
KR 207, Opp. Circuit House, Nayapura, Kota, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_120_19

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  Abstract 


Anterior cervical hypertrichosis (ACH) is a very rare form of primary localized hypertrichosis. It is characterized by a tuft of terminal hairs on the anterior neck just above the laryngeal prominence. Although it frequently presents with an isolated defect, it can be associated with other abnormalities. Here, we report the case of a 12-year-old girl who presented to our outdoor with a complaint of localized excessive hairs over the anterior aspect of the neck. She also gave a history of the persistence of irregular hyperpigmented areas on the right cheek. The diagnosis of ACH with linear and whorled nevoid hypermelanosis was made. Their concurrence might be coincidental. So far, ACH associated with linear whorled nevoid hypermelanosis has not been reported in the literature.

Keywords: Anterior cervical hypertrichosis, laryngeal prominence, linear and whorled nevoid hypermelanosis, tuft of terminal hairs


How to cite this article:
Saini S, Kumar R, Yadav D. Anterior cervical hypertrichosis with linear and whorled nevoid hypermelanosis in a 12-year-old girl: A rare association. Indian J Paediatr Dermatol 2020;21:135-7

How to cite this URL:
Saini S, Kumar R, Yadav D. Anterior cervical hypertrichosis with linear and whorled nevoid hypermelanosis in a 12-year-old girl: A rare association. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 May 26];21:135-7. Available from: http://www.ijpd.in/text.asp?2020/21/2/135/281726




  Introduction Top


Anterior cervical hypertrichosis (ACH) or “hairy throat” is a rare form of localized hypertrichosis that presents with a tuft of terminal hairs on the anterior neck just above the laryngeal prominence.[1] Its etiology is not well known.[2] While linear and whorled nevoid hypermelnanosis is a rare hyperpigmentation disorder characterized by linear streaks along the lines of blaschko, it usually appears within the first 2 years of life.[3]


  Case Report Top


A 10-year-old female child has been reported to the dermatology outdoor with her mother. She presented with a small area of excessive hair over the anterior aspect of the neck. It was there since infancy. Her mother gave a history of her child having the same length of hairs since birth. She also complained of an irregular area of hyperpigmentation presented on the right cheek since birth. It was small in size at birth but slowly increased in irregular size, shape, and became more pigmented as the age advanced. There was no history of consanguinity. She was born with normal vaginal delivery. All her milestones were appropriate according to the age. There was no pathology found in any family member.

On examination, multiple black terminal hairs were observed on the anterior aspect of the neck just above the laryngeal prominence [Figure 1]. There was no hypertrichosis in other body regions. Dermoscopy (Dermlite 3 with ×10) was used to know the types of hairs that showed terminal hairs [Figure 2]. On the other hand, there was a well-defined irregular hyperpigmented patch present in a linear and nevoid pattern on the right cheek extending from the malar region to just above the right mandibular line [Figure 3]. Dermoscopy of ill-defined hyperpigmented patch was done, and it showed net-like pattern, multiple brownish rings, brown curved lines, and hypopigmented dots [Figure 4]. Laboratory investigations for hyperandrogenemia were normal. Ophthalmological and neurological examinations were performed to evaluate accompanying anomalies, found to be normal. Plain and lateral cervical radiographs revealed no underlying pathology. Ultrasonography of the whole abdomen and pelvis was done and did not find any polycystic pathology. Informed and written consent was obtained from her mother.
Figure 1: A tuft of terminal hairs on the anterior aspect of the neck (front and lateral view)

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Figure 2: Dermoscopy of tuft of hairs showed terminal hairs (Dermlite 3 with × 10)

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Figure 3: Ill-defined hyperpigmented patch is present in linear and wholed pattern with close-up view

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Figure 4: Dermoscopy of ill-defined patch shows hyperpigmentation in “net-like pattern” (a), multiple brownish rings (green arrow), curved lines (blue arrows), and hypopigmented dot (red arrow) (b). (Dermlite 3 with × 10)

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Based on the clinical findings, the diagnosis of ACH and linear whorled nevoid hypermelanosis was made.

We advised her laser treatment for hair removal. With the successive 2 sittings of 808 nm diode laser, there was an 80% reduction in hair density and length [Figure 5]. Parents were counseled about the course and prognosis of linear and whorled nevoid hypermelanosis (LWNH).
Figure 5: Reduction in hair length and density after 2 sittings of 808 nm diode laser

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  Discussion Top


Hypertrichosis is the term used for excessive hair growth anywhere in the body in either male or female. It should be distinguished from hirsutism, which is reserved for females who grow an excessive amount of terminal hairs in androgen-dependent sites.[4],[5] First, Trattner et al.[6] reported ACH in three patients, who were members of Arabic families with consanguineous marriage. Peripheral neuropathy and bilateral hallux valgus were present in all three patients, whereas one patient had bilateral optic nerve atrophy and macular degeneration. Later, Tsukahara and Kajii[7] reported it in seven members of a Japanese family with no pathology except for one patient who had turner syndrome.

ACH may be familial or sporadic[2] with familial cases inherited as autosomal dominant generally or recessive and x-linked dominant.[7],[8] It frequently occurs as a solitary defect (70%) but may accompany anomalies such as peripheral sensory and motor neuropathy, hallux valgus, ophthalmological anomalies, mental retardation, and hypertrichosis on back.[9] ACH classified under congenital localized hypertrichosis may be observed in association with other cutaneous abnormalities such as congenital nevocellular nevi, Becker's nevi, linear epidermal nevi, congenital smooth muscle hamartomas, or familial hypertrichosis of the palms and soles.[10] Our case was found sporadic as there was no positive family history.

LWNH is a rare hyperpigmentation disorder characterized by streaks and swirls of macular hyperpigmentation along Blaschko's lines.[3] Although its causes are not well known, there is the possibility of developmental somatic mosaicism leading to the proliferation and migration of two mixed populations of melanocytes with different potentials for pigment production.[11] Naveen and Reshme[12] reported a “net-like” pattern of pigmentation, whereas Errichetti et al.[13] described brownish rings, curved lines, brownish streak-like lines, and focally distributed hypopigmented dots corresponding to perifollicular areas, as dermoscopic findings of linear and wholed nevoid hypermelanosis.

As ACH may present with other cutaneous abnormalities, it has not been reported with LWNH yet, so comment cannot be done on its pathomechanism. It could be explained as there might have been some somatic mosaicism or can present coincidently. To the best of our knowledge, this is most probably the first case report of sporadic ACH being presented together with LWNH in the English literature.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Echeverría XP, Cárdenas CP, Nicklas CA, Romero WA. Familial anterior cervical hypertrichosis. Indian J Dermatol Venereol Leprol 2010;76:579-81.  Back to cited text no. 1
    
2.
Monteagudo B, Cabanillas M, de las Heras C, Cacharrón JM. Isolated anterior cervical hypertrichosis. Actas Dermosifiliogr 2009;100:61-4.  Back to cited text no. 2
    
3.
Di Lernia V. Linear and whorled hypermelanosis. Pediatr Dermatol 2007;24:205-10.  Back to cited text no. 3
    
4.
Mimoto MS, Oyler JL, Davis AM. Evaluation and treatment of hirsutism in premenopausal women. JAMA 2018;319:1613-4.  Back to cited text no. 4
    
5.
Nguyen KD, Osehobo E, Alleyne CH. Hypertrichosis lanuginosa acquisita associated with intracranial melanoma: Case illustration. J Neurosurg 2018;129:48-9.  Back to cited text no. 5
    
6.
Trattner A, Hodak E, Sagie-Lerman T, David M, Nitzan M, Garty BZ. Familial congenital anterior cervical hypertrichosis associated with peripheral sensory and motor neuropathy – A new syndrome? J Am Acad Dermatol 1991;25:767-70.  Back to cited text no. 6
    
7.
Tsukahara M, Kajii T. Hairy throat: A dominant trait affecting seven members of a family. Clin Dysmorphol 1992;1:165-7.  Back to cited text no. 7
    
8.
Lee HW, Lee MW, Choi JH, Moon KC, Koh JK. Familial anterior cervical hypertrichosis. J Am Acad Dermatol 2005;53:530-2.  Back to cited text no. 8
    
9.
Nalluri R, Gilmour E, Brooke R. Anterior cervical hypertrichosis. Eur J Dermatol 2010;20:393-4.  Back to cited text no. 9
    
10.
Peñas PF, Jones-Caballero M, Amigo A, Aragüés M, García-Diéz A. Cutaneous meningioma underlying congenital localized hypertrichosis. J Am Acad Dermatol 1994;30:363-6.  Back to cited text no. 10
    
11.
Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol 1993;129:1460-70.  Back to cited text no. 11
    
12.
Naveen KN, Reshme P. Linear and whorled nevoid hypermelanosis with dermatoscopic features. Dermatol Online J 2014;20. pii: doj_21760.  Back to cited text no. 12
    
13.
Errichetti E, Pegolo E, Stinco G. Linear and whorled nevoid hypermelanosis: A case report with dermoscopic findings. Indian J Dermatol Venereol Leprol 2016;82:91-3.  Back to cited text no. 13
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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