Anterior Cervical Hypertrichosis: a Case Report

Case Report DOI: 10.6003/jtad.16104c8 Anterior Cervical Hypertrichosis: A Case Report

Mustafa Aksoy,1 MD, Yavuz Yeşilova,2* MD, Hacer Altın Sürücü,1 MD, Enver Turan,1 MD, Osman Tanrıkulu,1 MD

Address: 1Harran University School of Medicine, Department of Dermatology, Sanliurfa, 2Special Lokman Physician Hospital, Dermatology Clinic, Van, Turkey E-mail: [email protected] * Corresponding Author: Dr. Yavuz Yesilova, Special Lokman Physician Hospital, Dermatology Clinic, Van, Turkey

Published: J Turk Acad Dermatol 2016; 10 (4): 16104c8 This article is available from: http://www.jtad.org/2016/4/jtad16104c8.pdf Keywords: Anterior cervical hypertrichosis, hirsutism

Abstract

Observation: Anterior cervical hypertrichosis is a dermatosis of unknown cause, seen on the front of the neck, which is characterised by an increase in terminal hairs. It is generally sporadic and may be accompanied byanomalies such as peripheral and central neuropathy, mental retardation, retarded development, hallux valgus, ocular anomalies (optic atrophy, chorioretinal changes, hypermetropia), kyphoscoliosis, spina bifida, facial dysmorphism, conjoined muscles (synophyrys), lumbosacral or dorsal hypertrichosis and Turner syndrome. In this paper, the case is presented of an 11-year old male patient diagnosed with sporadic anterior cervical hypertrichosis.

Introduction which was first described in 1991 by Trattner et al. It is characterised by terminal hair on The terms of hypertrichosis and hirsutism are the anterior side of the neck [1, 2]. While it is often confused. However, these terms are often seen in the form of hypertrichosis alone, used to describe different situations. Hypert- various anomalies may accompany ACH. In richosis, the cause of which is generally unk- this paper, the case is presented of an 11- nown, is an increase in hair which occurs year old male patient diagnosed with sporadic independently of androgeny. There are no ACH. symptoms of oligomenorrhea, amenorrhea or virilisation (male-type hair on the temples, shrinkage of breast tissue, muscle tissue de- finition, clitoris growth and voice deepening). Hirsutism is hairiness which occurs with excessive androgeny. There is an increase in thick, dark hairs in body areas associated with androgen hormone, such as the upper lip, chin, around the breast, the lower abdo- men, the groin and thighs [1].

Anterior cervical hypertrichosis (ACH) is a ra- Figure 1. It is characterised by terminal hair on the rely seen congenital localised hypertrichosis, anterior side of the neck

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Case Report It is characterised by excessive hair on the posterior cervical vertebrae. Occasionally, it An 11-year old male presented at our polyclinic with the complaint of hair on the anterior side of may be determined together with kyphosco- the neck which had been ongoing for 1 year. liosis [5]. There was no anamnesis of any use of topical or ACH is hair seen in the area of the laryngeal systemic medication, trauma or inflammation be- prominence in particular. Often, no underl- fore the onset of the hairiness. No family member ying reason can be determined. Despite au- had any similar complaint. tosomal dominant transference, autosomal In the dermatological examination, there were recessive [6] and X dominant [7] forms have seen to be long, brown terminal hairs in an area also been described. The vast majority of re- of approximately 5 x 6 cm diameter (Figure 1). ported patients are familial [1]. Just as ACH No other dermatological findings were determined. The systemic examination and laboratory may be congenital, it is generally determi- test results were normal. No pathology findings ned as sporadic, as in the current case. were determined in the evaluations made by the Although not previously reported in Turkey, neurology and ophthalmolofy specialists. Treat- there have been 41 ACH patients reported ment of laser epilation was planned for the future in literature. The reported cases are gene- because of the patient’s age. rally female and determined at birth or in early childhood (n=31). In the majority of Discussion cases there was no other finding (n=32, 78%). In 9 patients (22%), there were vari- Hypertrichosis is classified in different ways ous other anomalies. Peripheral and central [3]. According to the etiology it may be idio- neuropathy was determined in 5 patients, pathic or acquired; according to the age at mental retardation in 2, and retarded deve- onset, it may be congenital or acquired; ac- lopment in 2 [8]. Other anomalies determi- cording to the area involved, it may be clas- ned in ACH are hallux valgus, ocular sified as localised or generalised anomalies (optic atrophy, chorioretinal hypertrichosis [3, 4]. Localised congenital changes, hypermetropia), kyphoscoliosis, hypertrichosis is characterised by well-defi- spina bifida, facial dysmorphism, conjoined ned excessive hair growth in any area of the muscles (synophyrys), lumbosacral or dor- body. It often has autosomal recessive sal hypertrichosis and Turner syndrome [1, transfer. There are 4 sub-types as lumbo- 8]. sacral hypertrichosis, hypertrichosis cubiti, The pathogenesis of ACH has not been pro- posterior cervical hypertrichosis and ACH ven and no genetic cause has been determi- [5]. ned. In recent studies, the hair follicles of Lumbosacral hypertrichosis (faun tail) is the ACH patients have been determined to show most frequently seen form of localised con- significant plasticity. Focus has been direc- genital hypertrichosis. Increased hair ted on an extended anagen phase or exces- growth in the sacral mid region is determi- sive growth of the hair follicles leading to an ned at birth. It is often seen together with abnormal signal pathway in ACH disease anomalies such as hyperpigmentation, sac- [9]. Treatments may include trimming, wa- ral dimple, spinal dysraphism, lipoma, ha- xing, bleaching, physical and chemical de- martoma, port-wine stain, dermatoid cyst, pilation, electrolysis, intense pulsed light sinus tract and aplasia cutis [5]. (IPL) and laser [1, 10]. Hypertrichosis cubiti (hairy elbow ACH causes both social and psychological syndrome), which occurs at birth or in in- problems for the patient. Although a benign fancy, is hypertrichosis seen on the extensor disease, ACH may sometimes accompany surfaces of the wrists. This sub-type of lo- neurological, ocular and orthopaedic ano- calised congenital hypertrichosis is bilateral malies. Therefore, in all patients diagnosed and seen together with anomalies such as with ACH a thorough clinical examination short height or facial asymmetry. Posterior (skin, neurology and ophthalmology exami- cervical hypertrichosis is associated with nations) and radiological evaluations must autosomal dominant or X recessive transfer. be made.

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