Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

Baliga, Vidya; Gopinath, V.; Baliga, Sudhindra; Ramachandra, Srinivas Sulugodu; Jithendra, K D; Shetty, Shamila

doi:10.5005/jp-journals-10024-1399

Cited by 3 publications

(3 citation statements)

References 11 publications

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“…9 Clínicamente en la mayoría de los casos el NS se presenta al nacimiento, como una placa blanda, superficie lisa, serosa o ligeramente verrugosa, sin pelo, redonda (Caso 1), lineal (Caso 2), que tiende a seguir las líneas de Blaschko, u oval (Caso 3), de color amarillento-naranja a rosado, variando en tamaño entre 1 y 10 cm, localizado en piel cabelluda, cara, cuello y raramente tronco, usualmente única, pero varias lesiones también han sido reportadas. 2,9 Raramente, anormalidades intraorales asociadas al NS han sido descritas, principalmente lesiones benignas desde papilomas de la mucosa bucal, encías, lengua y paladar, hasta anomalías dentales, usualmente contiguas a un NS de la cara, 13,14 y excepcionalmente como NS de mucosa oral sin afectación cutánea. 15 Las lesiones tienden a cambiar con el tiempo, particularmente durante la pubertad, cuando las lesiones tienden a ser más elevadas, verrugosas y grasosas debido al desarrollo pilosebáceo-apocrino, como ocurrió con el caso 1, caso 2 y caso 3.…”

Section: Discussionunclassified

Nevo sebáceo en la pubertad: serie de casos y revisión de la literatura

Lizardo-Castro,

Zavala-Sierra

2023

Rev Méd Hondur

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Antecedentes: El nevo sebáceo es un hamartoma, con una combinación de anomalías de la epidermis, folículos pilosos, glándulas sebáceas y apocrinas, de etiología desconocida y con igual incidencia en ambos sexos. Por lo general ocurre desde el nacimiento, localizado principalmente en piel cabelluda y cara, como placa alopécica, amarillo-naranja, de variable tamaño y forma. El aspecto clínico e histopatológico típicamente cambia con la edad, siendo máximo en la pubertad, por la influencia hormonal. En algunos casos se asocia con manifestaciones extracutáneas, como afectación del sistema nervioso central, ojos, sistema esquelético y riñones constituyendo el síndrome del nevo sebáceo. Los tumores secundarios originados sobre el nevo sebáceo son principalmente benignos. La extirpación quirúrgica de espesor total es el tratamiento de elección, pero existe controversia sobre la extirpación profiláctica y, la edad óptima de realizarla. Descripción de serie de casos: se presentan 4 pacientes pediátricos, atendidos en el Hospital Escuela, con lesiones localizadas en piel cabelluda y cara. Tres casos congénitos, manifestados como placa alopécica verrugosa, con rápido crecimiento en la pubertad y desarrollo de verruga vulgar en uno de ellos; el cuarto caso, presentó lesión verrugosa supraciliar derecha a los 14 años. En todos, la histopatología confirmó el diagnóstico. Conclusiones: Usualmente los nevos sebáceos son congénitos y solo presentan afectación cutánea. Su curso es rápido crecimiento en la pubertad, por influencia hormonal, y las neoformaciones sobre el nevo, raramente resultan malignas. Sugerimos la extirpación quirúrgica de los nevos sebáceos, con afectación cosmética y sin manifestaciones extracutáneas, antes de la pubertad.

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Section: Discussionunclassified

Nevo sebáceo en la pubertad: serie de casos y revisión de la literatura

Lizardo-Castro,

Zavala-Sierra

2023

Rev Méd Hondur

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“…Types of lesions. Oral manifestations of NSS include mucosal hyperpigmentation (47), papillomatosis, eventually causing soft tissue overgrowth on teeth (54,(56)(57)(58)(59)(60)(61)(62)(63)(64), and enamel defects (26), furthermore, hemihypertrophy and fibrous lesions (27,50,(59)(60)(61)(62) and enlarged papillae fungiformes of the tongue (33), uvula bifida (60), retention of teeth (26) and tooth position anomalies (33), numerical aberrations and dysmorphia of teeth (54,59,(63)(64)(65)(66), bone cyst of jaw (59), giant cell granuloma of the jaw (27,60) or 'osteoclastoma' (39), odontoma (67), and further odontogenic neoplasms such as ameloblastoma (35,45,68), ameloblastic fibro-odontoma (69), and adenomatoid odontogenic tumor (44,63,64). Basal cell carcinoma (BCC) is a feature of NSS, e.g., of lips (50).…”

Section: Nomenclature Nevus Sebaceous Syndrome (Nss) Ormentioning

confidence: 99%

Oral HRAS Mutation in Orofacial Nevus Sebaceous Syndrome (Schimmelpenning-Feuerstein-Mims-Syndrome): A Case Report With a Literature Survey

Friedrich

Gosau

Luebke

et al. 2021

In Vivo

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Background/Aim: The aim of this study was to present the long-term course of a patient with nevus sebaceous syndrome (NSS). Recent genetic studies place the syndrome in the emerging group of so-called RASopathies. The focus of the report is on surgical treatment and morphological and genetic findings of the face and oral cavity. Case Report: A female patient was treated for congenital alterations of facial skin and oral mucosa. The oral lesions were removed repeatedly. Eruption of teeth on the lesion sites was made easier by the measures taken. However, after repeated ablation of the affected gingiva, the periodontal papillomatous epithelium redifferentiated into the same reddish, conspicuous, hyperplastic epithelium. The teeth in the affected region showed noticeable changes in position, surface, and shape. A HRAS mutation was detected only in the regions of altered oral epithelia and not in adjacent soft tissues. Conclusion: Reports on NSS rarely address oral manifestations. The recorded alterations of oral soft and hard tissues in NSS indicate a topographical relationship between the development of oral mucosa and teeth as well as the long-lasting impact of a sporadic mutation on organ development at this site.Epidermal nevi (EN) are mosaic cutaneous developmental disorders (1-6). EN are differentiated according to the predominant epithelial cell types (7). The terminology of nevi refers to the histological components of the lesion (8). If extracutaneous findings are noted in addition to EN in an individual, this constellation is referred to as epidermal nevus syndrome (ENS) (1-6, 9-12). Some authors have used the term 'ENS' to describe a distinct entity, in particular (linear) nevus sebaceous syndrome (NSS) (synonymous: Schimmelpenning-Feuerstein-Mims (SFM) syndrome, Naevus sebaceous Jadassohn (syndrome), Solomon syndrome, and further terms) (1-6, 8, 10). Other authors subsumed a group of cutaneous diseases characterized by nevi and extracutaneous findings under the term 'ENS' (1, 10). Currently, the term 'ENS' is mostly used as an umbrella designation for several syndromes describing diseases with a potentially large variety of physical findings that frequently first attract attention through noticeable alterations in superficial skin layers, in particular nevi (3)(4)(5)8). With this broad sense, ENS also includes nevi that have adnexal differentiation (10). 274This article is freely accessible online.

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“…• Alterations of periodontal tissues to plaque in presence of certain systemic diseases has been reported • Hence emphasis on oral hygiene maintenance in such patients is extremely essential • They should be evaluated periodically as they are prone to develop tumors [25]…”

Section: Dsmentioning

confidence: 99%

Syndromes Affecting Periodontium

Vandana

Reddy

Desai

et al. 2015

Journal of Advanced Oral Research

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Syndrome is group of signs and symptoms associated with any morbid process and constituting together the picture of the disease. This poses a great challenge to the examiner to adequately diagnose and distinguish a syndrome from other pathologies. Although many of these disorders are not preventable, or curable early detection may allow significant improvement in the health care. Dentist is often in a unique position to pick up a previously unrecognized genetic or birth defect problem in a patient or family. Since many syndromes affect the oral structures in the unique way it aids in diagnosis. Although curing of genetic diseases is not yet available, maintenance of good oral health is important.

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Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome

Cited by 3 publications

References 11 publications

Nevo sebáceo en la pubertad: serie de casos y revisión de la literatura

Nevo sebáceo en la pubertad: serie de casos y revisión de la literatura

Oral HRAS Mutation in Orofacial Nevus Sebaceous Syndrome (Schimmelpenning-Feuerstein-Mims-Syndrome): A Case Report With a Literature Survey

Syndromes Affecting Periodontium

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