A 4-year-old female child born out of non-consanguineous marriage presented with multiple, oval-shaped hairless areas over the left parietal area and vertex on the scalp [Figure 1]; brownish papules coalescing to form plaques along the lines of Blaschko over the left side of face and neck, suggestive of Blasckhoidal epidermal nevi; and ipsilateral limbal dermoid on the sclera since birth [Figure 2]. There was no history of psychomotor disability or family history of the same. Dermoscopy of scalp lesions showed “golf-club set-like” appearance and absent hair follicles[1] [Figure 3]. The parents denied a biopsy.
Figure 1.
Well-demarcated atrophic scars of 0.5–1 cm over the scalp predominantly involving the left parietal area and vertex with a hair collar sign
Figure 2.
Brown-colored papules along lines of Blaschko involving the left side of the face extending up to the neck and ipsilateral painless nodule over sclera suggestive of limbal dermoid (red arrow)
Figure 3.
Dermoscopy of scalp lesions revealed prominent vessels (black arrow) with radially arranged hair shafts with visible elongated hair bulbs and darkly pigmented proximal ends through the semitranslucent epidermis was present (star-burst-like or golf club set-like appearance) (red arrow), absent hair follicle in center of the lesion (blue arrow)
Diagnosis
Aplasia cutis congenita (ACC) type 3.
Aplasia cutis congenita (ACC) type 3 is a congenital condition associated with epidermal nevi and organoid nevi, while psychomotor and ophthalmologic abnormalities may or may not be present. Sathishkumar et al. proposed that scalp ACC with epidermoid nevi could be mosaic RASopathy.[2] The parents were counseled regarding neurological abnormalities and advised for regular follow-up. The child was referred to a neurologist; MRI brain was normal. The ophthalmological evaluation did not reveal any significant findings other than limbal dermoid.
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 The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.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.
Acknowledgment
Department of Pediatrics and Department of ENT, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India
References
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