Nevus Comedonicus Syndrome: A New Pediatric Case

Abstract

Nevus comedonicus (NC) is an uncommon developmentaldefect 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.

Further delineation of the phenotypic spectrum of nevus comedonicus syndrome to include congenital pulmonary airway malformation of the lung and aneurysm

“… Note : Search details included: (“naevus”[All Fields] OR “nevus, pigmented”[MeSH Terms] OR (“nevus”[All Fields] AND “pigmented”[All Fields]) OR “pigmented nevus”[All Fields] OR “nevus”[All Fields] OR “nevus”[MeSH Terms]) AND comedonicus[All Fields] AND (“syndrome”[MeSH Terms] OR “syndrome”[All Fields]). References: (Kaliyadan, Nampoothiri, Sunitha, & Kuruvilla, ; Patrizi, Neri, Fiorentini, & Marzaduri, ; Pavithra, Pai, Mallya, & Pai, ; Ferrari et al, ; Suite and Mahabir, ; Seo, Piao, Suhr, Lee, & Park, ; Yadav, Mendiratta, Rana, & Chander, ; Qian, Liu, Zhou, & Zhang, ; Engber, , ; Whyte, ; Ghelbazouri et al, ; Alpsoy, Durusoy, Ozbilim, Karpuzoğlu, & Yilmaz, ; Ito, Mitamura, Tsuji, Harada, & Urabe, ; Vidaurri‐de la Cruz, Tamayo‐Sánchez, Durán‐McKinster, de la Luz Orozco‐Covarrubias, & Ruiz‐Maldonado, ; Martinez, Levrero, Bazzano, Larre Borges, & De Anda, ; Filosa, Bugatti, Ciattaglia, Salaffi, & Carotti, ). NC has been seen in patients with dual diagnoses such as orofacial digital syndrome (Baker & Agim, ) and Alagille syndrome (Woods, Larcher, & Harper, ).…”

The group of epidermal nevus syndromes

“…Cataracts that are usually localized ipsilaterally are a characteristic feature, 85,133-137 and many patients have bone defects. 85,129,[138][139][140] Less frequently reported anomalies include various neurologic defects, 85,141 widely spaced nipples, 85 ipsilateral corneal erosion, 142 and spinal dysraphism. 143 For differential diagnosis, the syndrome of segmentally arranged basaloid follicular hamartomas with ipsilateral osseous, dental, and cerebral anomalies may be considered.…”

Successful treatment of late‐onset nevus comedonicus with Er:YAG laser

“…It is a rare hamartoma caused by a developmental defect involving the pilosebaceous unit, with a predilection of face and neck area . In 50% of NC cases, the lesions appear shortly after the birth, and in the majority of the cases lesions develop before the age of 10 . It rarely appears in adulthood, only single case reports of development of NC in adulthood have been presented .…”