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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Linear nevus sebaceus syndrome is characterized by the association of nevus sebaceus covering extensive areas on the head and scalp with abnormalities of the central nervous system, ophthalmologic and skeletal changes, and malignancies. The incidence is approximately one per 10,000 live births, and there is no sexual predilection reported. The original description of this syndrome was followed by extensive literature describing the dermatologic, neurologic, and ophthalmologic manifestations of this disease. The objective of this report is to describe the surgical approach for the excision and reconstruction of giant sebaceous nevi of the face and scalp in children with linear nevus sebaceus syndrome on the basis of a consecutive series of five patients treated over 10 years in the same institution. To the authors' knowledge, this report represents the largest surgical series and suggests a reliable approach to the treatment of the cutaneous manifestations of this syndrome.
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PMID:Surgical management of the cutaneous manifestations of linear nevus sebaceus syndrome. 1475 90

Linear epidermal naevus (LEN) in the genital area is quite rare. It may present at birth or appear later on in life, in infancy or childhood and occasionally for the first time in adult life. There are several variants of epidermal naevi (EN), which, to the less experienced, can be mistaken for warts. When extensive, it can be associated with abnormalities in other organ systems (epidermal naevus syndrome). The definitive treatment of LEN is surgical ablation with excision of underlying dermis, but this frequently leads to scarring. Laser therapy is an alternative treatment modality and good results have been shown. We report an unusual case of LEN in the genital area in a 60-year-old man presenting as genital warts.
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PMID:A case of linear epidermal naevus presenting as genital warts--a cautionary tale. 1582 31

Linear nevus sebaceous is a hamartomatous lesion most commonly seen on the face and head of infants and diagnosed after birth. We describe a patient with sonographic findings of linear nevus sebaceous seen initially at 30 weeks' gestation. Other associated sonographic findings include macrocephaly and polyhydramnios. When associated with a midline soft tissue mass, these findings are highly suggestive of the presence of congenital linear nevus sebaceous sequence.
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PMID:Sonographic characteristics of linear nevus sebaceous sequence. 1628 5

Linear nevus sebaceous syndrome is reported to occur in as many as 1 in 1000 live births, and is hypothesized to result from genetic mosaicism involving a lethal autosomal-dominant gene. The terms "epidermal nevus syndrome" and "linear nevus sebaceous syndrome" are often used interchangeably, although linear nevus sebaceous syndrome more strictly applies to patients with a typical midline nevus, and epidermal nevus syndrome is a more inclusive term that includes all varieties of epidermal nevi. Linear nevus sebaceous syndrome encompasses a broad spectrum of abnormalities that may affect every organ system, including the central nervous system. In these cases, seizures and mental retardation are the main manifestations. Many other organ systems were also reported to be involved in this syndrome, including the cardiovascular, skeletal, ophthalmologic, and urogenital systems, among others. Although linear nevus sebaceous syndrome occurs at a relatively high frequency and may affect different organ systems, many physicians are unaware of the syndrome, which may delay diagnosis and treatment. We present two cases of linear nevus sebaceous syndrome, and we describe their initial presentation and subsequent evolution, incorporating a review of the current literature in this field.
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PMID:Linear nevus sebaceous syndrome: case reports and review of the literature. 1827 57

Linear epidermal nevus is an uncommon diagnosis of benign lesions of the oral cavity. It is characterized by a congenital malformation arising from the ectoderm cells, which are arranged according to a typical linear configuration known as Blaschko's lines. We report a case of linear epidermal nevus of oral cavity in a 51-year-old lady or woman. The linear epidermal nevus of the oral cavity, although rare, can be considered a differential diagnosis of oral papillomatosis (OP). The histopathological studies and detailed description are the center of the diagnostic and clinical evolution.
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PMID:Linear epidermal nevus of the oral cavity: a rare diagnosis. 2281 16

Linear nevus sebaceous syndrome is a multisystem disorder including nevus sebaceous, which is the hallmark of this syndrome, together with central nervous system, ocular and skeletal anomalies. We report a case of extensive skin lesions, CNS and eye anomalies in a full term infant who was conceived by intracytoplasmic sperm injection.
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PMID:Linear nevus sebaceous syndrome in a neonate conceived by intracytoplasmic sperm injection. 2402 11

A 20-year-old man presented to the oral and maxillofacial surgery department for evaluation and treatment of a dentofacial deformity. On examination, it was noted that he had marked hemifacial hyperpigmented papillomatous lesions covering the upper and middle thirds of the right side of his face extending to the right upper lid. Of note, he also had extensive intraoral involvement. These features were highly suggestive of linear epidermal nevus, a congenital hamartomatous lesion of the ectoderm. Linear epidermal nevus with oral manifestations is extremely rare with very few cases reported in the medical literature.
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PMID:Linear Epidermal Nevus: A Case Report With Oral and Facial Manifestations. 2716 Mar 64

Linear epidermal nevus is a congenital malformation characterized by linear, often one-sided arrangement. The lesions are localized along the Blaschko's lines, are present at birth, or appear in early childhood. They can be single or multiple, and have various colors-from skin color to dark brown. These lesions persist through the whole life making a significant cosmetic defect. Here, we present three clinical cases of epidermal nevus treated with CO2 laser. In a female patient, verrucous, dark brown skin eruptions were observed at the back of earlobe and down the neck. In the cases of the male patients, the lesions were located in the area of the neck and left blade. The removal of nevi was performed in stages, by cutting and evaporation using a CO2 laser. A very good therapeutic effect was obtained. CO2 laser treatment is the method of choice for the removal of extensive epidermal nevi. It is characterized by high efficacy and safety.
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PMID:Linear verrucous epidermal nevi-effects of carbon dioxide laser therapy. 2718 76

Linear nevus sebaceous syndrome (LNSS) is characterized by nevus sebaceous, mental retardation, seizures, and ocular abnormalities such as complex limbal choistoma. A young male with history of mass in right eye and blackish discoloration of skin over right and left side of forehead since birth presented with foreign body sensation and diminished vision in right eye. Ocular examination showed mass over epibulbar region with chorioretinal coloboma and posterior staphyloma in right eye and megalocornea in left eye. Histopathology report revealed complex limbal choristoma with compound melanocytic nevus. The case was managed by surgical excision of the limbal mass and filling the gap with scleral graft.
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PMID:Complex limbal choristoma in linear nevus sebaceous syndrome managed with scleral grafting. 2785 26

Nevus unius lateris is a rare congenital hamartoma derived from the ectoderm, considered to be a systematized verrucous variant of an epidermal nevus. Due to its extensive unilateral distribution, it is frequently associated with neurological, musculoskeletal, auditory, and visual abnormalities. A case report of a 25-year-old female patient with a diagnosis of nevus unius lateris without associated comorbidities is presented.
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PMID:Nevus Unius Lateris: A Case Report. 3124 58


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