Unilateral Follicular Facial Papules in a Child

INTRODUCTION A 12-year-old boy, born of nonconsanguineous marriage, came with a 2-year history of gradually progressing, asymptomatic hyperpigmented lesion on the left cheek. He gave no history of any topical application, photoexacerbation, atopy, or similar history among family members. On examination, there were closely set follicular papules on a hyperpigmented background on the left cheek [Figure 1]. Further, the hair, nails, and mucosa appeared normal. Dermoscopy revealed round whitish areas with keratotic follicular plugs surrounded by gray spots on a reddish brown background [Figure 2]. Histopathological examination showed follicular plugging with hyperkeratosis, hyperpigmentation of the basal layer, and dermal vasodilation [Figure 3].Figure 1: Left cheek showing multiple 2 mm sized closely set follicular keratotic papules with hyperpigmentation in the interfollicular areasFigure 2: Whitish areas showing follicular plugging with bluegrey dots over a reddish brown background (dermoscopy, ×50)Figure 3: Follicular plugging, increased basal layer pigmentation with dermis showing minimal perivascular inflammatory infiltrate and vasodilatation (H and E, ×100)What is your diagnosis? DISCUSSION Final diagnosis: Erythromelanosis follicularis faciei Erythromelanosis follicularis faciei (EFF) was first described by Kitamura et al. in 1960.[1] It is considered as a rare subtype of keratosis pilaris characterised by a clinical triad of sharply demarcated patches showing hyperpigmentation, erythema and closely set follicular papules with follicular plugging. It usually affects the preauricular areas and cheeks which gradually extend to involve the neck hence termed as EFF et colli (EFFC).[2] In the darker skin types, erythema may not be apparent to the naked eye. In such cases, dermoscopy is a valuable noninvasive tool aiding in the diagnosis of EFFC. It shows round whitish areas with follicular plugs, some of which are centered by a hair with surrounding areas showing blue gray spots or peppering in a reddish-brown background.[3,4] The follicular papules correspond to whitish areas with keratotic plugs on dermoscopy and follicular hyperkeratosis on histology. The erythema and hyperpigmentation correspond to blue gray spots with reddish brown background on dermoscopy and basal layer hypermelanosis with vasodilatation and pigment incontinence on histology.[3] Unusual features of our case were the unilateral presentation with erythema inapparent to naked eye and absence of keratosis pilaris elsewhere. This case also highlights the utility of dermoscopy in the diagnosis of EFFC, so that biopsy can be avoided as it involves the face. 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.