Nevus Comedonicus Syndrome

Dear Sir/Mam, An 11-year-old boy presented to the dermatology outpatient department with multiple mildly itchy black dots over the right side of the neck present since birth. The patient also had a history of few episodes of generalized seizure, the cause of which could not be ascertained at that time. The patient also had undergone corrective surgery for club foot in the past. There was no history of similar lesions in the family. On examination, there were multiple, grouped open comedones over the right side of the neck. At the margin of this, there were three subcutaneous bluish nodules of size 0.2 cm × 0.3cm topped with comedones. In the same area, there were two tender erythematous nodules of size 0.5 cm × 0.5 cm and 1.5 cm × 1.5 cm, respectively, with larger one showing surface ulceration, crusting, and peeling of the skin (since last 10 days) [Figure 1]. There were two asymptomatic, well-defined, light-brown-colored flat lesions of size 0.5 cm × 0.5 cm and 2.5 cm × 1 cm over the back and medial aspect of the left upper arm, respectively, which were suggestive of café-au-lait macules (CALMs) [Figure 2a and b]. The ocular examination did not show any abnormality. The X-ray of the whole spine showed thoracic scoliosis [Figure 3]. Clinical diagnosis of nevus comedonicus (NC) syndrome with furunculosis was considered based on morphology and other extracutaneous manifestations. The disease condition and prognosis were explained to the parents, and he was treated for furuncle with oral amoxicillin and clavulanic acid.Figure 1: The neck area showing multiple, grouped open comedones, bluish subcutaneous nodules, and two erythematous nodules with larger one showing surface ulceration, crusting, and peeling of the skinFigure 2: Well-defined, light-brown-colored macules (café au lait macules) over (a) Back area and (b) Medial aspect of the left upper armFigure 3: The X-ray of the whole spine anteroposterior and lateral view showing thoracic scoliosisNC or comedo nevus is a rare type of epidermal nevus, presenting as closely arranged, grouped, often linear, slightly elevated papules that have at their center keratinous plugs. It is caused by a defect in the development of the follicular infundibulum.[1] It may present at birth or appear later in life. The head-and-neck areas are the common sites. This may appear as a cluster of comedones or a single giant lesion.[2] Two types have been described – noninflammatory/nonpyogenic NC with acne-like characteristics and inflammatory type characterized by the formation of cysts, papules, pustules, and abscesses in various stages.[3] Our case was of inflammatory type. The term NC syndrome indicates comedo nevus presenting in association with extracutaneous manifestations, which may include central nervous system, skeletal, ophthalmological, neurological, and spinal abnormality.[4] We herein report the case of a child with NC syndrome with associated skeletal and neurological abnormalities in addition to CALMs. CALMs have been described in association with several genetic syndromes such as neurofibromatosis, McCune–Albright syndrome, tuberous sclerosis, Fanconi anemia, and Bloom syndrome. Our patient does not match the clinical features of any of the abovelisted syndromes. Unilateral or bilateral congenital cataracts can present as ocular manifestations in NC syndrome.[5] However, this case did not have any ophthalmological findings. We report this case because of its rarity. Our case was unusual as our patient also had associated CALMs which have not yet been described in association with NC syndrome to the best of our knowledge. Declaration of patient 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.