Abnormalities epidermal nevus syndrome nevus sebaceous of jadassohn. Medical search. Frequent questions

Usually, no symptoms of the nevi are present, with the exception of inflammatory linear verrucous epidermal nevus. (medscape.com)
Inflammatory linear verrucous epidermal nevus is a linear, persistent, pruritic plaque, usually first noted on a limb in early childhood. (medscape.com)
Inflammatory linear verrucous epidermal nevus, unlike the other types of epidermal nevi, demonstrates erythema and sometimes pruritus. (medscape.com)
Inflammatory linear verrucous epidermal nevus is characterized by tiny, discrete, erythematous, slightly warty papules, which tend to coalesce in a linear formation. (medscape.com)
Inflammatory linear verrucous epidermal nevus may occur with musculoskeletal abnormalities in a few children, prompting the classification of inflammatory linear verrucous epidermal nevus as part of epidermal nevus syndrome. (medscape.com)
One infant had inflammatory linear verrucous epidermal nevus with congenital dislocation of the ipsilateral hip and Fallot tetralogy of the heart. (medscape.com)
Another infant had congenital inflammatory linear verrucous epidermal nevus with congenital bony anomalies of the ipsilateral extremities. (medscape.com)
Nevus depigmentosus and inflammatory linear verrucous epidermal nevus may occur together, as may inflammatory linear verrucous epidermal nevus and melanodontia. (medscape.com)
These include linear sebaceous nevus, linear nevus comedonicus (NC), linear epidermal nevus (LEN), and inflammatory linear verrucous epidermal nevus (ILVEN). (medscape.com)
[ 5 ] delineated inflammatory linear verrucous epidermal nevus as a distinct entity in 25 patients. (medscape.com)
They coined the name inflammatory linear verrucous epidermal nevus, labeling it a clinical and histopathologic type of linear verrucous nevus that is often inflammatory or psoriasiform. (medscape.com)
Inflammatory linear verrucous epidermal nevus accounts for approximately 5% of patients with epidermal nevi and has been described in a mother and daughter. (medscape.com)

This neurocutaneous disorder is characterized by a craniofacial nevus sebaceus that falls along embryonic cutaneous lines and tends to be associated with neurological, ocular, skeletal, and vascular abnormalities. (medscape.com)
3. Extensive cerebriform nevus sebaceus: an unusual presentation. (nih.gov)
4. Nevus marginatus: a distinct type of epidermal nevus or merely a variant of nevus sebaceus? (nih.gov)
5. Epidermodysplasia verruciformis-associated and genital-mucosal high-risk human papillomavirus DNA are prevalent in nevus sebaceus of Jadassohn. (nih.gov)
11. Squamous cell carcinoma with perineural involvement in nevus sebaceus. (nih.gov)
17. [Jadassohn's nevus sebaceus and nevus unius lateris. (nih.gov)
18. Cutaneous leiomyosarcoma, trichoblastoma, and syringocystadenoma papilliferum arising from nevus sebaceus. (nih.gov)

Nevus comedonicus syndrome has ocular, skeletal, and central nervous system anomalies. (medscape.com)
Epidermal nevus syndrome" is sometimes used as a synonym, but more often as a broader term referring to Schimmelpenning syndrome in addition to nevus comedonicus syndrome, CHILD syndrome, Becker's nevus syndrome, and phakomatosis pigmentokeratotica. (wikipedia.org)
Cataracts may be a prominent feature of nevus comedonicus syndrome. (medscape.com)

Linear sebaceous nevus, also known as organoid nevus syndrome, often has the term linear deleted because almost all syndromic sebaceous nevi are linear. (medscape.com)
Organoid nevus syndrome is a general term that could be applied to at least five different types of ENS. (skincarehealthcenter.com)

A has been reported with varying levels of somatic mosaicism in individuals affected with anomalous pancreaticobiliary ductal union, epidermal nevus and Keratinocytic epidermal nevi and Schimmelpenning syndrome characterized by nevus sebaceous and extracutaneous abnormalities (Examples: Shimotake_2003, Bourdeaut_2010 and Hafner_2012, Groesser_2012). (varsome.com)

Schimmelpenning syndrome is a neurocutaneous condition characterized by one or more sebaceous nevi, usually appearing on the face or scalp, associated with anomalies of the central nervous system, ocular system, skeletal system, cardiovascular system and genitourinary system. (wikipedia.org)
The classic Schimmelpenning syndrome diagnosis comprises a triad of sebaceous nevi, seizures, and mental retardation. (wikipedia.org)
Since the original identification of Schimmelpenning syndrome, the number of findings has expanded to the point that the syndrome is associated with a considerable constellation of abnormalities. (wikipedia.org)
The most common structural central nervous system abnormalities in Schimmelpenning syndrome are hemimegalencephaly and ipselateral gyral malformations. (wikipedia.org)
Schimmelpenning syndrome appears to be sporadic rather than inherited, in almost all cases. (wikipedia.org)
There is no test to determine whether an individual born with a sebaceous nevus will go on to develop further symptoms of Schimmelpenning syndrome. (wikipedia.org)
hence, the term Schimmelpenning syndrome. (medscape.com)
It has also been called Schimmelpenning-Feuerstein-Mims syndrome and Jadassohn nevus phakomatosis. (medscape.com)
Schimmelpenning syndrome, as noted above, links a sebaceous nevus with cerebral anomalies, coloboma, and lipodermoid of the conjunctiva. (medscape.com)
Endocrine abnormalities such as vitamin D-resistant rickets have been associated with Schimmelpenning syndrome. (skincarehealthcenter.com)
In the recent past, the term was used to denote a specific disorder now known as Schimmelpenning syndrome. (skincarehealthcenter.com)
In one family this variant was found in an infant with severe Schimmelpenning syndrome, whereas the monozygotic twin brother was unaffected showing that this variant in the affected individual was due to a postzygotic somatic event (PMID: 22683711, 23255105). (varsome.com)

In 84% of patients with linear sebaceous nevus, skin lesions are on the face, and in approximately 50%, lesions are on the scalp, the neck, and the forehead. (medscape.com)
A syndrome characterized by lesions occurring on the face, scalp, or neck which consist of congenital hypoplastic malformations of cutaneous structures and which over time undergo verrucous hyperplasia. (nih.gov)
6. Rapidly growing pigmented tumor on a scalp nevus sebaceous of a pediatric patient: Observation or excision. (nih.gov)
19. [Developmental abnormalities and nevi of the scalp]. (nih.gov)
Epidermal nevi often occur on the trunk, limbs, face or scalp. (skincarehealthcenter.com)

Skeletal abnormalities may include dental irregularities, scoliosis, vitamin D-resistant rickets and hypophosphatemia. (wikipedia.org)
The choice of imaging studies depends on the suspected abnormalities and might include skeletal survey, CT scan of the head, MRI, and/or EEG. (wikipedia.org)
Additionally it is associated with neurological symptoms and skeletal, ophthalmological, urogenital, and cardiovascular abnormalities. (nih.gov)
Linear epidermal nevus syndrome is a congenital neurocutaneous disorder characterized by linear epidermal nevus with significant involvement of the nervous, ophthalmologic, and/or skeletal systems. (medscape.com)
Epidermal nevus syndromes (ENSs) are a group of rare complex disorders characterized by the presence of skin lesions known as epidermal nevi associated with additional extra-cutaneous abnormalities, most often affecting the brain, eye and skeletal systems. (skincarehealthcenter.com)
Skeletal abnormalities can include abnormal curvature of the spine, malformation of the hip and abnormalities of the arms and legs (e.g., underdevelopment or absence or overgrowth of limbs). (skincarehealthcenter.com)

This article discusses epidermal or keratinocyte nevi, nevus sebaceous, nevus comedonicus, smooth muscle hamartomas, and inflammatory linear verrucous epidermal nevi. (nih.gov)
Epidermal nevi have also been classified as "hamartomas", a rather vague and ambiguous term for benign tumor-like malformations that can affect any area of the body. (skincarehealthcenter.com)

Solomon defines epidermal nevus syndrome as a sporadic neurocutaneous linkage of congenital ectodermal defects in the skin, brain, eyes, and/or skeleton. (medscape.com)
Epidermal nevi are usually present at birth (congenital) and appear as a patch or plaque of overgrown skin. (skincarehealthcenter.com)

The abnormalities may occur in a variety of combinations, and need not include all three aspects of the classic triad of sebaceous nevus, seizures and mental retardation[citation needed]. (wikipedia.org)
Sebaceous nevi occur in 1 to 3 of 1000 births, with equal incidence by sex. (wikipedia.org)

The major ocular abnormalities are colobomas and choristomas. (wikipedia.org)
[ 1 ] In 1957, he described a case of sebaceous nevus involving the head, with ipsilateral ocular lesions including coloboma of the upper lid, increased density of cranial bones, epileptic seizures, and mental retardation. (medscape.com)
Ocular abnormalities may include cataracts, clouding (opacity) of the cornea or partial absence of tissue of the iris or retina (colobomas). (skincarehealthcenter.com)

In most patients, the lesions appear on 1 side of the body (nevus unius lateralis), as shown in the images below. (medscape.com)
The term organoid nevus may be used to emphasize the admixture of epidermal cells often evident in individual lesions of epidermal nevi. (medscape.com)

This variant has been reported in the literature as a somatic event (present in tissue from a lesion but not in non-lesional tissue or peripheral blood)Â¬â€ in individuals with nevus sebaceous syndrome (PMID: 23255105, 22683711, 23096712, 26521233). (varsome.com)
It was also observed in a child with epidermal nevus, polycystic kidneys, rhabdomyosarcoma and growth retardation (PMID: 20805368). (varsome.com)

Generally, epidermal nevi on the head and face are more likely to be associated with malformations of the brain, eyes and cranial bones. (skincarehealthcenter.com)

found: seizures in 67% of cases intellectual disability in 61% of cases ophthalmological abnormalities in 59% of cases involvement of other organ systems in 61% of cases structural abnormality of cerebrum or cranium in 72% of cases The major neurological abnormalities include intellectual disability to varying extent, seizures, and hemiparesis. (wikipedia.org)
Neurological abnormalities that can be associated with ENSs can include seizures, cognitive impairment, developmental delays and paralysis of one side of the body (hemiparesis). (skincarehealthcenter.com)

2. An update on Becker's nevus: Pathogenesis and treatment. (nih.gov)
7. Becker's nevus. (nih.gov)
8. Intense pulsed light treatment for Becker's nevus. (nih.gov)
9. Clinicopathologic characteristics of early-onset Becker's nevus in Korean children and adolescents. (nih.gov)
10. Becker's nevus syndrome. (nih.gov)
20. Segmental Becker's nevi with mucosal involvement. (nih.gov)

The cell types that live in the epidermis include epidermal cells or keratinocytes, sebaceous glands, hair follicles, apocrine and eccrine glands, and smooth muscle cells. (nih.gov)

Nevus comedonicus is evident clinically as confluent clusters of dilated follicular orifices plugged with keratin, giving the appearance of aggregated open comedones. (medscape.com)

A different variant affecting the same residue G12S is associated with Cardio-facio-cutaneous syndrome in HGMD (Nava_2007). (varsome.com)

In this review, 4 distinct epidermal nevus syndromes, recognizable by the different types of associated epithelial nevi, are described. (medscape.com)
Therefore, the umbrella term "epidermal nevus syndromes" now represents a group of distinct disorders that have in common the presence of one of the various types of epidermal nevi. (skincarehealthcenter.com)

It has been reported that up to 10% of individuals with epidermal nevi may develop additional syndrome symptoms, but that number appears to be inconsistent with the rarity of the syndrome and may be overstated. (wikipedia.org)
What are the symptoms for epidermal nevus syndrome? (skincarehealthcenter.com)

Epidermal nevi are overgrowths of structures and tissue of the epidermis, the outermost layer of the skin. (skincarehealthcenter.com)

Epidermal nevus syndrome is often termed the Solomon syndrome. (medscape.com)

Individuals with a sebaceous nevus of the head may later develop intralesional tumors such as trichoblastoma or basal cell carcinoma. (skincarehealthcenter.com)

The earlier in embryological development such a mutation occurs, the more extensive the nevi are likely to be and the greater the likelihood of other organ system involvement. (wikipedia.org)

However, it is inappropriate to use these terms to denote a single disorder or interchangeably with epidermal nevus syndromes. (skincarehealthcenter.com)
Joseph Merrick, the so-called "elephant man", apparently suffered from Proteus syndrome and not NEUROFIBROMATOSIS , a disorder with similar characteristics. (bvsalud.org)

Seizures are reported in 75% of patients with linear sebaceous nevus, mostly appearing in the first 6 months of life. (medscape.com)
Linear nevus comedonicus is also known as comedone nevus, nevus follicularis keratosus, nevus acneiformis unilateralis, and nevus zoniform. (medscape.com)

As with other epidermal nevi, the most common sites are the face, the trunk, and proximal extremities. (medscape.com)
Epidermal nevi on the trunk are more likely to be associated with abnormal curvature of the spine, hip malformation and deformities of the arms and legs. (skincarehealthcenter.com)

Epidermal Nevi and Related Syndromes - Part 2: Nevi Derived from Adnexal Structures. (nih.gov)

The term "epidermal nevus syndrome" has generated significant controversy and confusion in the medical literature. (skincarehealthcenter.com)
However, the term epidermal nevus syndrome could be correctly applied to several different disorders. (skincarehealthcenter.com)

A clinical entity called epidermal nevus syndrome should be more precisely defined and distinguished by clinical, histopathologic, and genetic criteria. (medscape.com)

Prevalence is unknown, but Epidermal nevus syndrome is listed with the National Organization for Rare Disorders, which defines rare as affecting "fewer than 200,000 people in the United States. (wikipedia.org)
Epidermal nevus syndromes encompass a wide variety of disorders. (skincarehealthcenter.com)

Nevi or nests of cells may be made up of a variety of cell types. (nih.gov)
The different types of epidermal nevi can vary in size, number, location, distribution and appearance. (skincarehealthcenter.com)
There are different types of epidermal nevi based upon their main component. (skincarehealthcenter.com)

Characteristic epidermal nevus in the axillary fossa of a child with Jadassohn nevus phakomatosis. (medscape.com)

treatment may include potential surgical removal of the nevus. (wikipedia.org)

Each type may be regarded as part of a syndrome with systemic associations. (medscape.com)

Diseases17

Chemicals and Drugs1

Inflammatory linear verrucous epidermal nevus12

Sebaceus7

Comedonicus syndrome3

Organoid nevus syndrome2

Keratinocytic1

Schimmelpenning12

Scalp5

Skeletal6

Hamartomas2

Congenital2

Occur2

Ocular3

Lesions2

PMID2

Cranial1

Neurological2

Becker's6

Apocrine1

Dilated follicular1

Cutaneous1

Distinct2

Symptoms2

Epidermis1

Solomon1

Individuals1

Involvement1

Disorder2

Linear2

Trunk2

Structures1

Term2

Clinical1

Disorders2

Types3

Child1

Include1

Associations1