Radiographic features of the linear nevus sebaceous syndrome

Leonidas, J C; Wolpert, S M; Feingold, Murray; McCauley, RG

doi:10.2214/ajr.132.2.277

Cited by 31 publications

(22 citation statements)

References 11 publications

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“…Several craniofacial abnormalities have occurred in ENS, including: skull asymmetry, ''fibrous dysplasia,'' premature closure of the sphenofrontal suture, deformities of the sphenoid bone, skeletal hypertrophy as well as hypoplasia, and bony irregularities in addition to absence of cranial bone [Schimmelpenning, 1957[Schimmelpenning, , 1983Marden and Venters, 1966;Leonidas et al, 1979;Martinez and Gimenez, 1985;Vles et al, 1985;Rogers et al, 1989;Fritzsch et al, 1995;Olivares et al, 1999]. Abnormalities of the central nervous system, which are frequently ipsilateral to nevi on the scalp, have been reported [Leonidas et al, 1979;Levin et al, 1984;Vles et al, 1985;ElShanti et al, 1992;Fritzsch et al, 1995;Allison et al, 1998]; however, alterations of the skull in these regions are rarely described.…”

Section: Discussionmentioning

confidence: 99%

Skeletal changes in epidermal nevus syndrome: Does focal bone disease harbor clues concerning pathogenesis?

Heike

Cunningham

Steiner

et al. 2005

American J of Med Genetics Pt A

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Epidermal nevus syndrome (ENS) is a rare, sporadic, congenital disorder of unknown etiology featuring a complex and highly variable phenotype that can include focal or generalized skeletal disease. We describe a young man with ENS manifesting right-sided linear skin lesions, generalized weakness, diffuse osteopenia associated with hypophosphatemic rickets, and distinctive focal bone lesions ipsilateral to the skin findings. Review of the literature concerning ENS-associated skeletal disease suggested such focal bone defects are fibrous dysplasia, but our patient did not have the typical radiographic or histopathologic findings of fibrous dysplasia. Nevertheless, his circulating fibroblast growth factor 23 (FGF-23) level was elevated, likely functioning as a "phosphatonin," yet no activating mutations in GNAS previously reported in fibrous dysplasia or McCune-Albright syndrome were detected in his leukocytes or affected skin. We postulate that the focal skeletal disease, although different than fibrous dysplasia, may be a source of FGF-23 in ENS.

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Section: Discussionmentioning

confidence: 99%

Skeletal changes in epidermal nevus syndrome: Does focal bone disease harbor clues concerning pathogenesis?

Heike

Cunningham

Steiner

et al. 2005

American J of Med Genetics Pt A

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“…LNDD first described by Schimmelpenning and subsequently by Feuerstein and Mims. (4) Both nevi and syndrome have been described under many names -Jadassohn nevus phacomatosis, nevus unius lateris, organoid nevus syndrome, Solomon syndrome. (2) The incidence of sebaceous nevi at birth is estimated at 1 in 1000, but prevalence of these lesions in association with other anomalies as in case of LNSS varies between case reports to around 1 in 10,000 (6-orphanet).…”

Section: Discussionmentioning

confidence: 99%

“…(2) Linear sebaceous nevus first described by Jadassohn in 1895. (4) A paramedian location nevus is typically vertical in orientation.…”

Section: Discussionmentioning

confidence: 99%

“…(5)(6) Abnormalities affecting other parts of body are connective tissue, skeletal system, cardiac and ocular abnormalities. Ocular abnormalities occur in 33% of patients with LNSS (4) and include coloboma of lid, iris and chorioretinal colobomas, generalised retinal degeneration, antimongoloid lid fissure, asymmetry of orbit bone. (3)(7) Cardiac anomalies include coarctation of aorta and ventricular septal defect.…”

Section: Discussionmentioning

confidence: 99%

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Linear Nevus Sebaceous Syndrome - Radiological Findings

Ranjan¹,

Priyadarshi²

2016

jemds

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“…9 The epidermoid or linear nevus sebaceous is present over the head and neck. 10 It is a triphasic lesion, which is bland in infancy, has papillomatous hyperplasia in adolescence, and associated with skin-appendage tumors in adults.…”

Section: Special Imaging Casebookmentioning

confidence: 99%

Hemimegalencephaly and Linear Nevus Sebaceous Syndrome

Herman

Siegel

2001

J Perinatol

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A full-term infant boy was born at term by elective Caesarean section after a pregnancy complicated by an intrauterine sonogram at 18 weeks, which demonstrated a right intracranial cyst. The child was noted to have somatic asymmetry with right hemihypertrophy and a large head. A slight yellowish hyperpigmented linear skin lesion was present in the midline along the nose extending to the lip. The child had frequent seizures during the first 2 weeks of life, which became too numerous to count and difficult to control. The seizures consisted of brief eye flutter or whole body tonic-clonic seizures. The child became progressively more lethargic and did not feed well. Cranial magnetic resonance imaging (Figure 1) was performed. DENOUEMENT AND DISCUSSIONThe MRI of the head demonstrates the presence of hemimegalencephaly (HME) with an enlarged hemisphere with mass effect, but with a dilated lateral ventricle. A renal sonogram demonstrates asymmetric kidneys with the right being larger and containing small cysts. The cutaneous lesion was diagnosed as an epidermal nevus or linear nevus sebaceous. The child, therefore, is thought to have the linear nevus sebaceous syndrome (LNSS; Feuerstein-Mims syndrome) with HME.

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Radiographic features of the linear nevus sebaceous syndrome

Cited by 31 publications

References 11 publications

Skeletal changes in epidermal nevus syndrome: Does focal bone disease harbor clues concerning pathogenesis?

Skeletal changes in epidermal nevus syndrome: Does focal bone disease harbor clues concerning pathogenesis?

Linear Nevus Sebaceous Syndrome - Radiological Findings

Hemimegalencephaly and Linear Nevus Sebaceous Syndrome

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