Nevus comedonicus syndrome

BACKGROUND: Nevus comedonicus is a rare disease and diagnosis is made chiefly on clinical grounds. It may occur in isolation, or in rare cases, it may be combined with other congenital malformations to constitute nevus comedonicus syndrome. We report a case of this disease herein. PATIENTS AND METHODS: A young woman aged 20 years presented with cribriform plaques since birth resulting from the juxtaposition of depressed pigmented scars and comedonic lesions. These lesions were found on the entire face, the left hemithorax and the left upper limb, with well delineated distribution stopping at the median line. The patient's history revealed congenital cataract of the left eye operated at the age of 10 years. Clinical diagnosis of nevus comedonicus was confirmed by histological examination of the skin. Laboratory screening for other associated malformations revealed no abnormalities. Oral retinoids were proposed but the patient refused all treatments. DISCUSSION: Nevus comedonicus is one of the rarest forms of cutaneous nevus. This case is original as regards the extent and topography of the nevus covering the left side of the patient's body and its association with congenital cataract potentially indicating nevus comedonicus syndrome. This syndrome is extremely rare and belongs to a large group of epidermal nevus syndromes characterised by association of nevus and extracutaneous malformations, particularly ocular, skeletal and neurological.     

Nevus comedonicus with hidradenoma papilliferum and syringocystadenoma papilliferum in the female genital area

Background Nevus comedonicus is an uncommon malformation of the pilosebaceous unit. Association of nevus comedonicus with tumors originating from the apocrine gland has not previously been reported. Method We present a case of nevus comedonicus associated with hidradenoma papilliferum (HAP) and syringocystadenoma papilliferum (SCAP) in the female genital area. Results Histological features strongly suggested nevus comedonicus associated with HAP and SCAP. Conclusion Although the association of nevus comedonicus with HAP and SCAP is very rare, the possibility of coexistence cannot be excluded.     

Nevus comedonicus with epidermolytic hyperkeratosis

A 7-year-old girl had a linear nevus comedonicus affecting the right upper limb. Histologic examination of two biopsies specimens showed both the common changes of nevus comedonicus and the presence of epidermolytic hyperkeratosis. Therefore, nevus comedonicus should be included in the list of dermatoses having the distinctive histologic pattern of epidermolytic hyperkeratosis.     

Keratoacanthoma arising in nevus comedonicus

Nevus comedonicus is a benign hamartoma of the pilosebaceous unit and is considered as a rare subtype of epidermal nevus. It was first described in 1895. It manifests as a group of closely dilated follicular openings with dark keratin plugs resembling comedones. Malignant degeneration of a nevus comedonicus is exceptional; we report a case of keratoacanthoma arising in extensive nevus comedonicus.     

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.     

Epidermal nevus syndrome and didymosis aplasticosebacea

Abstract:  The epidermal nevus syndrome is a disease complex consisting of the association of an epidermal nevus with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular, and urogenital systems. The epidermal nevi are classified according to their predominant component; nevus sebaceus (sebaceous glands), nevus comedonicus (hair follicles), and nevus verrucosus (keratinocytes). We report a neonate who presented with a nevus sebaceus on the scalp and face as well as a coloboma and dermoid on his left eye. Within the sebaceous nevus on the scalp, circumscribed lesions of aplasia cutis congenita were detected, which is consistent with the recently proposed term in the literature didymosis aplasticosebacea.     

Cytokeratin and filaggrin expression in nevus comedonicus

To elucidate the pathogenesis of abnormal keratinization in nevus comedonicus, we performed an immunohistochemical study using antikeratin and antifilaggrin (filament-aggregating protein) antibodies. There were no significant differences between nevus comedonicus and normal skin in cytokeratin expression. Although filaggrin was only detected in the granular layer in open comedones, filaggrin was detected in both superficial cells and also intermediate cells in closed comedones, suggesting that filaggrin is involved in the formation of closed comedones. The disorder of terminal differentiation related to filaggrin may play a role in the pathogenesis of abnormal keratinization in nevus comedonicus.     

Nevus Comedonicus Syndrome—Nevus Comedonicus Associated with Ipsilateral Polysyndactyly and Bilateral Oligodontia

Abstract: A 7‐year‐old boy diagnosed as a case of nevus comedonicus syndrome is presented because of the rarity of the condition and the association of the previously unreported features oligodontia and unilateral polysyndactyly restricted to the same side as the nevus comedonicus.     

Nevus Comedonicus in Oral‐Facial‐Digital Syndrome Type 1: A New Finding or Overlapping Syndromes?

We report a patient with oral‐facial‐digital syndrome type 1 (OFDS1) who exhibited features overlapping those of nevus comedonicus syndrome, an unusual presentation that may potentially represent a new variant of OFDS1. OFDS1 and nevus comedonicus syndrome represent two rare syndromes with numerous overlapping features that have yet to be described in relation to one another. The features present in our patient led us to propose the possibility of a new variant of OFDS1 in which nevus comedonicus represents a cutaneous manifestation of the syndrome. Knowledge of this potential relationship is important for identification and management of the syndromes' accompanying manifestations in affected patients and may offer further insight into crossroads of pathogenesis.     

Nevus Comedonicus: A Case Series

Twelve previously unreported cases of nevus comedonicus are presented. Characteristic closely grouped dilated follicular openings with horny plugs that mimic comedones led to the diagnosis. One patient had nevus comedonicus syndrome and there were cases with atypical locations and unusual complications of this condition. We also highlight clinical associations and therapeutic options.     

Nevus comedonicus

The nevus comedonicus (NC) is an uncommon variant of adnexal hamartoma which appears clinically as linear groups of open comedones. Its name may be a misnomer since, according to some, true comedones are not present. NC usually occurs by itself but may be linked with a variety of systemic findings such as skeletal or ocular anomalies. Although the nevus comedonicus is viewed by many as a hamartoma arising from a defective mesoderm, others consider this lesion to be an epidermal nevus involving the hair follicle or an appendageal nevus of sweat ducts.     

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

Nevus comedonicus, a rare congenital hamartoma of the pilosebaceous unit, is characterized by keratotic plugging. It usually occurs after birth and during early childhood. It rarely appears in adulthood. Despite the benign nature of the condition, it usually requires treatment due to aesthetic reasons. Several treatments have been reported in nevus comedonicus, most of them resulting with recurrences. Here, we report a case of nevus comedonicus with adult onset, successfully treated with erbium‐doped yttrium aluminum garnet (Er:YAG) laser therapy.     

Extensive inflammatory nevus comedonicus involving half of the body

Nevus comedonicus is an uncommon variant of adnexal hamartoma without known cause. It usually occurs on the face, neck and chest and appears as groups of closely arranged dilated follicular openings with keratin plugs. We report extensive inflammatory nevus comedonicus in a 14-year-old boy involving half of his body, limited by the midline, with suppuration and residual scarring. The lesions first appeared at 3 years of age and worsened at the start of puberty. The clinical features, etiopathology, histopathology and treatment options of nevus comedonicus are briefly reviewed in the light of the literature.     

Extensive Nevus Comedonicus Involving the Palm: Questionable Role of the Pilosebaceous Unit in Pathogenesis

A 10‐year‐old boy had multiple grouped pits with black plugs arranged along the lines of Blaschko on his left chest, arm, and palm. Involvement of the palms is rarely reported in the literature. Recent reports have described mosaic acneiform conditions that could share pathogenetic mechanisms with nevus comedonicus. We briefly review the literature on mosaic conditions with acneiform lesions including nevus comedonicus.     

Epidermal nevus syndromes

The term "epidermal nevus syndrome" (ENS) has been used to describe the association of epidermal hamartomas and extra-cutaneous abnormalities. Epidermal nevi follow the lines of Blaschko. The majority of the extra-cutaneous manifestations involve the brain, eye, and skeletal systems. Several subsets with characteristic features have been delineated including the nevus sebaceous syndrome, Proteus syndrome, CHILD syndrome, Becker nevus syndrome, nevus comedonicus syndrome, and phakomatosis pigmentokeratotica. Epidermal nevi have been associated with benign and malignant neoplasms. Advances in molecular biology have revealed that the manifestations of ENS are due to genomic mosaicism. It is likely that the varied clinical manifestations of ENS are due in great part to the functional effects of specific genetic defects. Optimal management of the patient with ENS involves an interdisciplinary approach. Amelioration of the cutaneous features of ENS has been difficult but there have been advances, especially in the use of lasers.     

Porokeratotic eccrine ostial and dermal duct nevus

A case of porokeratotic eccrine ostial and dermal duct nevus (PEODDN) affecting all 4 extremities is described. Clinically, palmo-plantar lesions resembled those of nevus comedonicus and the remaining lesions those of linear verrucous epidermal nevus. Histologically, all lesions were identical with cornoid lamellae in the eccrine ostia and epidermal ducts.     

Epidermal nevus syndromes

The term "epidermal nevus syndrome" (ENS) has been used to describe the association of epidermal hamartomas and extra-cutaneous abnormalities. Epidermal nevi follow the lines of Blaschko. The majority of the extra-cutaneous manifestations involve the brain, eye, and skeletal systems. Several subsets with characteristic features have been delineated including the nevus sebaceous syndrome, Proteus syndrome, CHILD syndrome, Becker nevus syndrome, nevus comedonicus syndrome, and phakomatosis pigmentokeratotica. Epidermal nevi have been associated with benign and malignant neoplasms. Advances in molecular biology have revealed that the manifestations of ENS are due to genomic mosaicism. It is likely that the varied clinical manifestations of ENS are due in great part to the functional effects of specific genetic defects. Optimal management of the patient with ENS involves an interdisciplinary approach. Amelioration of the cutaneous features of ENS has been difficult but there have been advances, especially in the use of lasers.     

Congenital nevus comedonicus complicated by a hidradenitis suppurativa‐like lesion: Report of a childhood case

Nevus comedonicus is a rare, benign hamartoma of the pilosebaceous unit that may be isolated or part of the nevus comedonicus syndrome. Although rare in children, complication by hidradenitis suppurativa‐like lesions has been described. We present a report of a 9‐year‐old girl in whom surgical excision was curative, with a review of the relevant literature.     

Acneform lesions in Becker's nevus.

Three cases of Becker's nevus are described in which acneform lesions were confined strictly within the limits of the hyperpigmentation. The combined phenomenon has not been reported previously, and we conceive it to be either a rare variant of the anomaly or a still rarer coincidence of Becker's nevus and nevus comedonicus.     

Unilateral linear basal cell nevus

Clinical, histopathological, electron-microscopical and cytophotometrical features of a case of linear unilateral basal cell nevus are reported. Several publications exist on basaliomas originating in a nevus verrucosus or nevus comedonicus. Our case, however, is regarded as an abortive form of the basal cell nevus syndrome due to somatic mutation. Only some few comparable cases are documented in the literature.