Nevus comedonicus syndrome: a new pediatric case.

Abstract

Nevus comedonicus (NC) is an uncommon developmental defect of the pilosebaceous apparatus. The individual lesions are large comedones often arranged in groups or in a linear pattern. Nevus comedonicus syndrome (NCS) is a well-defined disorder within the large group of epidermal nevus syndromes. In patients suffering from NCS, the nevus is associated with noncutaneous abnormalities including skeletal defects, cerebral anomalies, and cataracts. We report a 9-year-old boy with open comedones in a linear pattern localized to the right cheek, clinodactyly and polydactyly of the first right finger, bilateral syndactyly of the second and third toes, and some depigmented hairs since birth.

Nevus comedonicus

“…NC is an uncommon lesion, occurring much less frequently than any epidermal nevus subtype [1]. Fewer than 200 cases have been described [7], although it is likely that many instances are not reported unless some unusual feature is present to warrant publication [8].…”

“…Fewer than 200 cases have been described [7], although it is likely that many instances are not reported unless some unusual feature is present to warrant publication [8]. There is no gender or racial predisposition, and while 50% of cases are evident at birth [1], lesions commonly make their initial appearance during childhood or even middle age. NC is not known to be a familial disorder.…”

“…It is evident clinically as confluent clusters of dilated follicular orifices plugged with keratin, giving the appearance of aggregated open comedones [1, 2]. It is also known as comedone nevus, nevus follicularis keratosus, nevus acneiformis unilateralis and nevus zoniforme.…”

Nevus comedonicus syndrome

“…Nevus comedonicus syndrome (NCS) is a well-defined disorder within the large group of epidermal nevus syndromes. [3] In patients suffering from NCS, the nevus is associated with noncutaneous developmental abnormalities, including ipsilateral cataract, skeletal malformation, CNS abnormalities, and trichilemmal cysts. [4] A differential diagnosis of atrophoderma vermiculatum can be considered but they usually present as reticulated depressions with narrow ridges of normal-appearing skin, giving a worm-eaten appearance.…”

“…Unlike nevus comedonicus, nevus comedonicus syndrome is a rare entity with very few reports[2367] in the literature that thus merits the documentation. Since the number of cases reported is relatively low in the literature and several such cases should be existing in communities, reporting of these would enhance the quantum of reported cases that would in turn naturally enable us to elucidate the full spectrum of the disease.…”