The group of epidermal nevus syndromes

Happle, Rudolf

doi:10.1016/j.jaad.2010.01.017

Cited by 207 publications

(182 citation statements)

References 234 publications

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“…1 Nevi associated with ENSs follow the lines of Blaschko, a notable exception to which is the CHILD syndrome, in which lateralization pattern reflects the functional origin of the postzygotic modulation of the inherited mutation. 1 Nevus sebaceous occurring as a part of nevus sebaceous syndrome may be differentiated histologically, by the presence of hyperplastic sebaceous glands. 1 Coexistence of nevus sebaceous and nevus philus may be seen in phacomatosis pigmentokeratotica.…”

Section: Discussionmentioning

confidence: 96%

“…1 Nevus sebaceous occurring as a part of nevus sebaceous syndrome may be differentiated histologically, by the presence of hyperplastic sebaceous glands. 1 Coexistence of nevus sebaceous and nevus philus may be seen in phacomatosis pigmentokeratotica. Ipsilateral cataract is the diagnostic clue in nevus comedonicus syndrome, whereas linear nevus covered with soft white hair distinguishes Angora hair nevus from other epidermal nevi (EN).…”

Section: Discussionmentioning

confidence: 99%

“…ENSs may be distinguished by the type of associated epidermal nevus and by the criterion of presence or absence of heritability. 1 The most common oral manifestations reported are localized or diffuse verrucous growths on the lips, palate, gingiva, buccal mucosa and tongue. 2 Other findings include hypertrophy of the tongue, cleft palate, high arched palate, bifid uvula 3 and various dental anomalies, such as hypodontia, unerupted teeth, malformed teeth and odontodysplasia.…”

Section: Introductionmentioning

confidence: 99%

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Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

Baliga¹,

Gopinath²,

Baliga³

et al. 2013

The Journal of Contemporary Dental Practice

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Aim:To report rare findings of oral and periodontal manifestations in a patient with Epidermal nevus syndrome (ENS). Background:The ENS describes the rare association of an epidermal nevus with abnormalities of central nervous system,ocular and skeletal abnormalities. Reports of oral involvement have been few. Also, most of the intraoral lesions have been reported in patients with nevi that do not fulfill the criteria for the diagnosis of ENS.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

Baliga¹,

Gopinath²,

Baliga³

et al. 2013

The Journal of Contemporary Dental Practice

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“…In LEN marked papillomatosis, hyperkeratosis, and acanthosis with elongation of rete ridges in psoriasiform pattern is noted. 3 The observation of epidermolytic hyperkeratosis on biopsy should prompt genetic counseling. The often devastating disfigurement of epidermolytic hyperkeratosis (EHK) results from mutations in keratins 1 and 10.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Currently, four major different types of ENS are recognized: linear sebaceous nevus, linear nevus comedonicus, linear epidermal nevus, and inflammatory linear verrucous epidermal nevus (ILVEN). 3 ENS is likely a continuum, as many authors include a wide-array of various other syndromes under its mantle. The presence of epidermal nevi should alert the clinician to possible abnormalities in the underlying organ systems.…”

Section: Case Reportmentioning

confidence: 99%

A profound case of linear epidermal nevus in a patient with epidermal nevus syndrome

Adams¹,

Athalye²,

Schwimer³

et al. 2011

J Dermatol Case Rep

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Background: Epidermal nevus syndrome is a multi-system disease with a wide spectrum of clinical presentation. Numerous specialists may be required to address its extra cutaneous manifestations.Main observations: We report a severe case of epidermal nevus syndrome involving the oral cavity, pharynx, and central nervous system in addition to disfiguring skin lesions.Conclusions: Dermatologists are in a unique position to first render the diagnosis of epidermal nevus syndrome for young patients and ensure appropriate follow-up. (J Dermatol Case Rep. 2011; 5(2):

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Congenital Naevi and Other Developmental Abnormalities Affecting the Skin

Kinsler

Sebire

2016

Rook's Textbook of Dermatology, Ninth Edition

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The understanding of congenital naevi has evolved rapidly in the last few years, with the discovery of many of the causative genes, and an increasing understanding of the phenotypic correlates of genotypic mosaicism in the skin. A new classification of all congenital naevi is proposed, based on phenotype, histology and genetics, which allows for the addition of new conditions as they are discovered, and for the regrouping of previously distinct disorders. The clinical features and extracutaneous associations of different congenital naevi are reviewed, as well as those of non‐naevoid developmental abnormalities of the skin.

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The group of epidermal nevus syndromes

Cited by 207 publications

References 234 publications

Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

A profound case of linear epidermal nevus in a patient with epidermal nevus syndrome

Congenital Naevi and Other Developmental Abnormalities Affecting the Skin

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