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

These include linear sebaceous nevus, linear nevus comedonicus (NC), linear epidermal nevus (LEN), and inflammatory linear verrucous epidermal nevus (ILVEN). (medscape.com)
Inflammatory linear verrucous epidermal nevus is a linear, persistent, pruritic plaque, usually first noted on a limb in early childhood. (medscape.com)
[ 3 ] 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)
The vitamin D analogue calcipotriol may be tried in each form of this disease, particularly inflammatory linear verrucous epidermal nevus. (medscape.com)
Usually, no symptoms of the nevi are present, with the exception of inflammatory linear verrucous epidermal nevus. (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)

NS become more prominent during puberty, reflecting sebaceous growth, often becoming verrucous. (aad.org)
Sebaceous nevi and verrucous epidermal nevi are closely related, and many authors regard them as variants. (medscape.com)

In this review, 4 distinct epidermal nevus syndromes, recognizable by the different types of associated epithelial nevi, are described. (medscape.com)
Happle R. Linear Cowden nevus: a new distinct epidermal nevus. (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)
Nevus comedonicus syndrome has ocular, skeletal, and central nervous system anomalies. (medscape.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)

Nevus sebaceous was first identified in 1895 by Jadassohn. (wikipedia.org)
First described by Jadassohn in 1895, nevus sebaceus (NS) is a common lesion, affecting 0.3% of newborns. (aad.org)
In 1895, Jadassohn first described nevus sebaceus (see the image below), a circumscribed hamartomatous lesion predominantly composed of sebaceous glands. (medscape.com)

1,2) Larger lesions in a Blaschkoid distribution are observed in the epidermal nevus syndrome known as the Schimmelpenning-Feuerstein-Mims syndrome (SFM), associated with extracutaneous abnormalities of the eyes (strabismus, colobomas, lipodermoids), central nervous system (structural brain abnormalities, seizures), and skeleton (craniofacial, limb deformities). (aad.org)
The term organoid nevus may be used to emphasize the admixture of epidermal cells often evident in individual lesions of epidermal nevi. (medscape.com)
[ 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)
No ideal medical therapy for the cutaneous lesions of epidermal nevus syndrome exists. (medscape.com)
Dermoscopy and confocal microscopy may be helpful in the clinical diagnosis of sebaceous lesions. (medscape.com)
Immunohistochemistry may be helpful in the differential diagnosis between benign and malignant sebaceous lesions. (medscape.com)

Phacomatosis pigmentokeratotica is characterized by the presence of multiple organoid nevi with sebaceous differentiation, a speckled lentiginous nevus, and skeletal and neurologic abnormalities. (medscape.com)
Vitamin D analogues may work by inhibiting epidermal proliferation, promoting keratinocyte differentiation, and/or exerting immunosuppressive effects on lymphoid cells. (medscape.com)
It inhibits epidermal proliferation, promotes keratinocyte differentiation, and has immunosuppressive effects on lymphoid cells. (medscape.com)
Sebaceous epithelioma (also known as sebaceoma) differs from sebaceous adenoma mainly in regards to the degree of differentiation. (medscape.com)
Adipophilin and periplipin are two such antigens associated with sebaceous differentiation. (medscape.com)
4] Syringocystadenoma papilliferum, formerly known as nevus syringocystadenomatous papilliferus, is a proliferating tumor with mostly apocrine differentiation but eccrine differentiation can also be seen. (ijcrr.com)

Familial naevus sebaceous of Jadassohn. (medscape.com)
Happle R, Konig A. Familial naevus sebaceus may be explained by paradominant transmission. (medscape.com)
Squamous cell carcinoma and basal cell carcinoma arising in a naevus sebaceous of Jadassohn: case report and literature review. (medscape.com)

Post-zygotic mutations in FGFR2 have also been identified in mosaic forms of acne, keratinocytic epidermal nevi, nevoid acanthosis nigricans/rounded and velvety epidermal nevus (RAVEN). (aad.org)
Igawa S, Honma M, Minami-Hori M, Tsuchida E, Iizuka H, Ishida-Yamamoto A. Novel postzygotic KRAS mutation in a Japanese case of epidermal nevus syndrome presenting with two distinct clinical features, keratinocytic epidermal nevi and sebaceous nevi. (medscape.com)
Ousager LB, Bygum A, Hafner C. Identification of a novel S249C FGFR3 mutation in a keratinocytic epidermal nevus syndrome. (medscape.com)
Keratinocytic epidermal nevus syndrome with Schwann cell proliferation, lipomatous tumour and mosaic KRAS mutation. (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)
Syringocystadenoma papilliferum is a benign adnexal tumor of the skin,[2] which can occur either de novo or on some organoid nevus such as nevus sebaceous. (ijcrr.com)

Other benign and malignant tumors include apocrine cystadenoma , leiomyoma and sebaceous cell carcinoma. (medscape.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)
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)
Skeletal changes in epidermal nevus syndrome: does focal bone disease harbor clues concerning pathogenesis? (medscape.com)

Rarely, other malignancies such as sebaceous carcinoma, microcystic adnexal carcinoma, and melanoma may complicate NS. (aad.org)
Deletions of the patched gene have been identified in nevus sebaceus and may be responsible for the predisposition to the development of basal cell carcinoma and other tumors in this lesion. (medscape.com)
Wu ZW, Shi WM, Sun Y, Li XJ, Song J. Cutaneous spindle cell squamous cell carcinoma in nevus sebaceous. (medscape.com)
Sebaceous carcinoma is an outright malignant neoplasm with prominent cellular pleomorphism and anaplasia. (medscape.com)
Well-differentiated sebocytes in small nests, deeply infiltrating the subcutaneous tissue, thus consistent with sebaceous carcinoma. (medscape.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)
Sebaceous nevi are sporadic and occur with equal frequency in males and females of all races. (medscape.com)

The most frequent benign tumors are trichoblastomas and syringocystadenoma papilliferum, occurring in less than 5% of nevus sebaceus. (medscape.com)
A case of syringocystadenoma papilliferum associated with nevus sebaceous in a 26 year old female patient in the nipple areolar region. (ijcrr.com)
A rare site of occurance of syringocystadenoma papilliferum arising in nevus sebaceous is described. (ijcrr.com)
These classical histological features favored a diagnosis of Syringocystadenoma Papilliferum associated with Nevus Sebaceous. (ijcrr.com)

Schimmelpenning syndrome appears to be sporadic rather than inherited, in almost all cases. (wikipedia.org)
Solomon defines epidermal nevus syndrome as a sporadic neurocutaneous linkage of congenital ectodermal defects in the skin, brain, eyes, and/or skeleton. (medscape.com)

It has also been called Schimmelpenning-Feuerstein-Mims syndrome and Jadassohn nevus phakomatosis. (medscape.com)
Discordance for Schimmelpenning-Feuerstein-Mims syndrome in monochorionic twins supports the concept of a postzygotic mutation. (medscape.com)

It is a benign congenital hamartoma related to abnormalities of the epidermis, sebaceous glands, sweat glands, and hair follicles. (aad.org)
Microscopy showed dilated, keratin filled infundibulum surrounded by numerous mature and immature sebaceous glands. (ijcrr.com)
It is defined by hyperplasia of the epidermis, hair follicles, sebaceous and apocrine glands. (ijcrr.com)

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)
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)
Schimmelpenning syndrome, as noted above, links a sebaceous nevus with cerebral anomalies, coloboma, and lipodermoid of the conjunctiva. (medscape.com)
Happle R. Gustav Schimmelpenning and the syndrome bearing his name. (medscape.com)

Sebaceous carcinomas are common on the eyelid and tend to present with pagetoid extension of atypical sebaceous cells in the conjunctiva or in the epidermis. (medscape.com)

Nevus sebaceous is a benign, congenital hamartoma of the skin, with a predilection for the scalp and less commonly occurs on the face, around the ears, neck or on the trunk. (ijcrr.com)
DISCUSSION Nevus sebaceous is a congenital hamartoma of the skin, first described by Jadassohn. (ijcrr.com)

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

The medical importance of a solitary nevus sebaceus relates to the description of both benign change and, in some cases, malignant neoplastic change. (medscape.com)
Rarely, malignant eccrine poromas, sebaceous carcinomas, and apocrine carcinomas have been reported to result in widespread metastases and death. (medscape.com)
The development of secondary malignant neoplasms within the nevus sebaceus is rare and occurs almost exclusively in adults. (medscape.com)

Nevoid hypertrichosis, diffuse lipoatrophy and epidermal nevus: a new syndrome? (medscape.com)

In nevus sebaceus, postzygotic somatic mutations may result in various clinical expressions of mosaicism. (medscape.com)

An epidermal nevus syndrome with cerebral involvement caused by a mosaic FGFR3 mutation. (medscape.com)

Vidaurri-de la Cruz H, Tamayo-Sanchez L, Duran-McKinster C, de la Luz Orozco-Covarrubias M, Ruiz-Maldonado R. Epidermal nevus syndromes: clinical findings in 35 patients. (medscape.com)

There is a major problem with the latter method in that mutations are seen in sebaceous tumors in patients without the syndrome. (medscape.com)

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

Nevus sebaceous is a rare benign tumor in children that usually presents with warty patches of hair loss on the scalp. (medscape.com)

[ 32 ] Also helpful may be demonstration of lipid contents in the vacuoles of the sebaceous cells in less differentiated carcinomas. (medscape.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)

Of newborns, 0.3% are affected by nevus sebaceus. (medscape.com)

The clinical presentation of nevus sebaceous and syringocystadenoma papilleferum varies widely but the histological appearance is uniform and characteristic, forming the basis of diagnosis. (ijcrr.com)

Histologic section of sebaceous adenoma showing a predominance of sebaceous cells with prominent cytoplasmic vacuoles. (medscape.com)

Linear nevus comedonicus is also known as comedone nevus, nevus follicularis keratosus, nevus acneiformis unilateralis, and nevus zoniform. (medscape.com)
Inflammatory linear verrucose epidermal nevus. (medscape.com)

Nevus sebaceus occurs with equal frequency in males and females of all races. (medscape.com)

Confocal microscopy revealed sebaceous lobules composed by clusters of ovoid cells with dark nuclei and bright, highly refractile glistening cytoplasm. (medscape.com)

As with other epidermal nevi, the most common sites are the face, the trunk, and proximal extremities. (medscape.com)

The authors concluded: "Our study confirms that most of the secondary neoplasms arising in association with nevus sebaceus are benign. (aad.org)

A review of 20 sebaceous tumors revealed dermoscopic features of radially arranged, elongated crown vessels surrounding opaque structureless yellow areas or yellow comedolike globules and branching arborizing vessels. (medscape.com)

INTRODUCTION Nevus sebaceous is a congenital skin lesion commonly seen by pediatricians, dermatologists, and plastic surgeons. (ijcrr.com)

thus if any change is seen within the nevus sebaceous, the patients should seek medical advice. (medscape.com)
Mall V, Heinen F, Uhl M, Wellens E, Korinthenberg R. CNS lipoma in patients with epidermal nevus syndrome. (medscape.com)
Likewise, a high proportion of sebaceous adenomas demonstrate loss of MSH-2 or MLH-1 , but very few of these patients will have MTS. (medscape.com)
Some studies have indicated that examination of the sentinel lymph node may help in detecting early metastasis and therefore may help staging Muir-Torre syndrome patients. (medscape.com)

Those cases with cystic degeneration are more likely associated with Muir-Torre syndrome (MTS). (medscape.com)

No cases in this series displayed features of SFM syndrome. (aad.org)

Nevus sebaceus appears to respond to hormonal influences, as the lesion can be raised at birth, become flattened in childhood, and become raised again during puberty. (medscape.com)

Anatomy1

Diseases19

Chemicals and Drugs1

Inflammatory linear verrucous epidermal nevus13

Verrucous2

Distinct epidermal nevus2

Comedonicus syndrome3

Individuals with epidermal nevi1

18953

Lesions6

Differentiation6

Naevus3

Keratinocytic4

Organoid nevus2

Benign and malignant1

Skeletal3

Carcinoma5

Occur3

Syringocystadenoma papilliferum4

Sporadic2

Feuerstein-Mims2

Glands3

Schimmelpenning7

Epidermis1

Congenital hamartoma2

Follicular1

Malignant3

Nevoid1

Postzygotic1

Cerebral1

Findings1

Mutations1

Solomon1

Scalp1

Carcinomas1

Involvement1

Newborns1

Clinical Presentation1

Prominent1

Linear2

Occurs1

Clusters1

Common1

Secondary1

Review1

Skin1

Patients4

Cystic1

Features1

Appears1