Rare Association of Congenital Triangular Alopecia with Vitiligo

Madam, Congenital triangular alopecia (CTA) is a type of nonscarring alopecia characterized by a triangular- or lancet-shaped area of hair loss on the frontotemporal region of the scalp.[1] It can either be present at birth or can develop during early childhood.[2] Dermoscopy, a noninvasive diagnostic tool, can aid in differentiating it from alopecia areata and scarring alopecia by showing the presence of vellus hair.[3] Rarely, other systemic disorders or malformation syndromes may be associated with this.[2] Here, we report a case of CTA associated with vitiligo. A 12-year-old male child presented to us with a complaint of a patchy area of hair loss on the left side of the scalp since birth. The patch was nonprogressive and asymptomatic since its appearance. There was no history of any injury or skin abnormality at the site of hair loss at birth. There was no reported history of similar hair loss in any family members. On physical examination, a well-defined triangular approximately 3 cm × 3 cm patch of alopecia was observed on the left temporal area of the scalp [Figure 1]. The surrounding hair appeared normal with no evidence of scarring or inflammation. The hair pull test at the periphery of the patch came out to be negative. On further skin examination, depigmented patches were visible on the lateral and medial aspect of the left foot that developed 1 month before presentation to us [Figure 2]. The depigmented patches were highlighted by Wood's lamp examination. He had no history suggestive of atopy. No history of autoimmune diseases was present in any family members. On dermoscopy examination of the alopecia patch, it was observed that the follicular openings appeared normal and the bald patch contained numerous vellus hair. In addition, terminal hairs were found in the surroundings [Figure 3]. Exclamation mark hair was absent. Typical history, clinical findings, and dermoscopy of the patch suggested a diagnosis of congenital temporal triangular alopecia with vitiligo. Counseling of the parents was done about the benign aspect of the CTA condition. Routine blood tests, thyroid profile, and blood sugar levels were within the normal range. Tofacitinib, an oral Janus kinase inhibitor, was prescribed at a dose of 5 mg twice daily after required investigations. The patient was monitored closely for any adverse reactions and laboratory tests were performed to assess liver and kidney function. After 2 months of treatment with tofacitinib, the patient reported an improvement in the extent and severity of his vitiligo lesion, with an increase in repigmentation [Figure 4] and he did not report any adverse effects during the course of treatment. However, there was no improvement in his alopecia patch.Figure 1: Well-defined triangular patch of alopecia on the left temporal area of the scalpFigure 2: Depigmented patches on the lateral and medial aspects of the left footFigure 3: On dermoscopic examination of the alopecia patch, normal follicular openings with numerous vellus hair were observed. In addition, terminal hairs were found in the surroundingsFigure 4: Vitiligo lesions after 2 months of treatmentCTA or Brauer nevus is a circumscribed, noncicatricial, noninflammatory form of alopecia with no clear known cause. In 1905, Sabouraud first described this.[4] It is considered a developmental defect, typically noticed in young children, usually between the ages of 2 and 4 years old as unilateral patches of hair loss or, less frequently, bilateral patches of alopecia in the frontotemporal region.[3] Around 54 cases have been reported so far.[5] It was believed to be congenital, but now, many researchers believe this to be an acquired disorder.[6] It is often sporadic but may affect several members of a family.[7] Many times it is misdiagnosed as aplasia cutis congenita, alopecia areata, tinea capitis, trichotillomania, and traction alopecia. Dermoscopy and histopathology would differentiate this entity from others. Normal follicular openings and vellus hairs in the patch of alopecia are usually seen upon dermoscopy. On histopathology, the absence of mature hair follicles and the presence of vellus hairs are usually found resembling features of androgenetic alopecia.[8] The child under observation exhibited the characteristic symptoms of CTA, which included a triangular-shaped hair loss pattern that had been present since birth, with a frontotemporal location. In addition, there was an absence of exclamation mark hair on dermoscopy, ruling out alopecia areata. Furthermore, the skin in the affected area appeared normal with no features of scarring. These collectively pointed towards the of CTA.[5] In up to 15% of cases, it is associated with other disorders such as congenital heart diseases, congenital dislocation of hip, bone, and teeth abnormalities, central nervous system abnormalities, and rarely, it can be a part of malformation syndromes such as phakomatosis pigmentovascularis, Dandy–Walker malformation, Down's syndrome, multiple lentigines, and café-au-lait patches.[8] Till date, there is no report suggesting the co-occurrence of vitiligo and CTA. There is no definite treatment for CTA. Many consider this as a cosmetic problem. Hair restoration surgery and complete excision of the lesions are among the available treatment options.[6] The use of tofacitinib in this child was effective in improving the vitiligo, but it was not effective in treating the alopecia. Although CTA is benign and remains asymptomatic throughout life and few cases have been reported, we report this case to highlight the rare association of CTA with vitiligo. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children 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.