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.     

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

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.     

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.     

The Nevus Comedonicus Syndrome: A case Report with Emphasis on Associated Internal Manifestations

A 14-year-old boy with nevus comedonicus, multiple congenital abnormalities of the spine, and a central nervous system disorder is described. The associated abnormalities previously reported with nevus comedonicus are summarized. Thorough evaluation of patients with nevus comedonicus may disclose the occurrence of certain internal abnormalities in syndromic association.     

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.     

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.     

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.     

Extensive Nevus Comedonicus with Inflammatory Nodules and Cysts Controlled with Adalimumab

Nevus comedonicus is a very rare skin disorder characterized by the presence of comedo-like dilated pores with keratinous plugs, rarely resulting in painful recurrent inflammatory nodules or cysts. It presents as localized or extensive form. It displays unilaterally or bilaterally segmental distribution. Histopathologically, it is characterized by keratin-filled epidermal invagination with bulbous proliferation of keratinocytes. The condition may be caused by fibroblast growth factor receptor 2 mutation. Although it may be controlled by a variety of therapeutic modalities, it is difficult to achieve complete resolution. We report a case of extensive nevus comedonicus with inflammatory nodules and cysts controlled with adalimumab.     

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.     

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.     

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.     

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.     

Nevus Comedonicus Bilateralis Et Verruciformis

A case of nevus comedonicus is described which was bilateral, more prominent on the right side, worse on the buttocks with unusual verrucous nodules on the right helix, knee and elbows, and affecting the palms and soles. This patient would appear to have been the most seriously afflicted of the 130 reported cases. In addition to the more comedo-like lesions on the trunk, a unique feature was the gradual development of verrucous nodules on the helix of the right ear, knee, hand and elbows. The pathologic changes on the nevus comedonicus on the trunk showed the epidermis to be invaginated, in some instances suggesting a tubular-like quality, with a dilated orifice containing keratin protruding above the surface. Sebaceous glands and hair shaft were absent. The pathologic changes of the verrucous nodule could be described as a more severe form of nevus comedonicus with folding and involution of the epidermis forming large crypts and cysts filled with keratin. Lesions were generally more severe in areas of pressure, which might explain the production of verrucous nodules.     

Epidermal nevus syndromes: clinical findings in 35 patients

Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.