|Year : 2018 | Volume
| Issue : 2 | Page : 78-80
Congenital triangular alopecia of occipital scalp: A rare entity
Naveen Kumar Kansal, Payal Chauhan
Department of Dermatology and Venereology, All Institute of Medical Sciences, Rishikesh, Uttarakhand, India
|Date of Web Publication||10-Jul-2018|
Naveen Kumar Kansal
Department of Dermatology and Venereology, All India Institute of Medical Sciences, Rishikesh - 249 203, Uttarakhand
Source of Support: None, Conflict of Interest: None
Congenital triangular alopecia (CTA), a relatively less recognized condition of the scalp, presents as a localized nonscarring patch of alopecia. The condition is likely to be confused with more common diseases for example alopecia areata. Recently, a case of occipital CTA presented to us, whose parents had applied indigenous topical medicine to cure the lesion with resultant irritant reaction. The aim of our case report is to increase awareness of the condition so that unnecessary treatments may be avoided.
Keywords: Brauer nevus, congenital triangular alopecia, nonscarring alopecia, trichoscopy
|How to cite this article:|
Kansal NK, Chauhan P. Congenital triangular alopecia of occipital scalp: A rare entity. Clin Dermatol Rev 2018;2:78-80
| Introduction|| |
Congenital triangular alopecia (CTA, also known as temporal triangular alopecia, Brauer nevus or hypotrichotic nevus) is a localized, nonscarring form of alopecia typically affecting the frontotemporal region and less commonly the temporoparietal scalp. Very few cases of CTA are reported on the occipital scalp. CTA can develop at birth or later in childhood as a triangular, oval- or lancet-shaped, nonprogressive patch of alopecia. We report a case of 2-year-old child who presented with a single patch of hair loss over the occiput since birth.
| Case Report|| |
A 2-year-old male child presented with a single fixed area of hair loss over the occipital scalp, which was present since birth. It was asymptomatic and nonprogressive in nature. The parents gave a history of topical application of some ayurvedic indigenous medication a week back which was followed by redness over the lesion and surrounding area. There was no history of absence of skin over the patch at birth. On examination, a single lancet-shaped 3 cm × 2 cm patch of nonscarring alopecia was present over occipital scalp [Figure 1]. Erythema and scaling were noted over the patch. Few vellus hairs were also present within the patch. There were no exclamation mark hairs. Trichoscopy could not be performed as child was very irritable. The presence of the lesion since birth, relative fixity of the size, shape of the lesion without any exclamation mark hairs led to a clinical diagnosis of CTA. The parents were counseled regarding the poor chances of hair growth with any topical treatment and to consider hair transplantation when the child grows.
|Figure 1: Single lancet-shaped, 3 cm × 2 cm size alopecic patch, with erythema and scaling, overlying the occipital scalp|
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| Discussion|| |
As already stated, CTA usually affects the frontotemporal, or less commonly the temporoparietal scalp. However, about 2.5% of cases present with the area of alopecia overlying the occipital scalp. Although termed as “congenital,” CTA usually manifests after the age of 2–3 years or later as the surrounding nonaffected vellus hair are gradually replaced by terminal hair. The condition appears to be quite rare, and since the first description of CTA in 1905, only 128 cases of this entity have been reported in literature till now.,, To the best of the authors' search, only two cases of occipital CTA have been reported in literature [Table 1].,, However, this “rarity” of the disorder has been questioned earlier as well  and certainly may be due to relative lack of awareness.
|Table 1: Characteristics of reported occipital congenital triangular alopecia cases|
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The diagnosis of CTA remains essentially clinical, depending on its location, appearance, and stability of the alopecia. More recently, trichoscopy (using a dermatoscope) has proved to be a useful diagnostic tool to clinically rule out the more common differentials such as alopecia areata, tinea capitis, trichotillomania, and traction alopecia. Furthermore, Karadag Köse and Güleç identified trichoscopic follicular features of short vellus hairs, vellus hair length diversity and white hairs in all their nine CTA patients. Histopathological examination, though not essential to diagnosis, shows unremarkable epidermis and dermis without any inflammatory infiltrate. Hair follicles are normal in number but miniaturized with increase in vellus or indeterminate hairs. Sweat and sebaceous glands show no abnormality. Common differential diagnoses, with differentiating features are described in [Table 2].
|Table 2: Common differential diagnoses of congenital triangular alopecia|
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CTA is associated with a number of other disorders and syndromes in about 15% of the cases. The disorders include café au lait macules, congenital cardiac diseases (valvular and septal defects) skeletal abnormalities (congenital dislocation of the hip, spina bifida), Dandy-Walker malformation, epilepsy and mental retardation, multiple lentigines, and tracheoesophageal fistula. The syndromic associations include chromosomal disorders (Down's syndrome, Turner syndrome), phakomatosis pigmentovascularis, Klippel–Trénaunay syndrome, LEOPARD syndrome More Details, and Pai syndrome. However, no disorder/syndrome has been reported with occipital CTA.,
CTA tends to be possibly missed clinically  and may erroneously be diagnosed as other causes of nonscarring alopecia like alopecia areata. This fact certainly may lead to inadvertent treatment attempts with topical and intralesional steroids. The clinicians should remain aware of this benign condition so that patient can be counseled adequately regarding the nature of the disease. This is essential to avoid the unnecessary investigations or attempts at potentially harmful treatment options to cure the disease, like in our case where the patients took indigenous treatment from outside leading to irritant reaction. Treatment with corticosteroids, either topical or intralesional steroids, is completely ineffective, though Bang et al. reported successful treatment of CTA with 3% topical minoxidil in a 1-year-old girl. However, the hair growth was not maintained in their case, and hair loss resumed after stopping the treatment. Surgical hair restoration procedures such as follicular unit transplantation or complete excision can be offered to the patient for acceptable cosmetic outcomes.,
Thus, we present this case to bring the focus to this uncommonly diagnosed and probably underreported disease and to differentiate it from other causes of nonscarring alopecia by its unique presentation and stable course. This can avoid injudicious treatment attempts thus minimizing the treatment-related adverse effects.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents had given their consent for images and other clinical information to be reported in the journal. They understand that names and initials of the patient will not be published and due efforts will be made to conceal patient's identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2]