Epidermal nevi and the epidermal nevus syndrome

Rogers, Maureen; McCrossin, Ian; Commens, Chris

doi:10.1016/s0190-9622(89)70061-x

Cited by 209 publications

(147 citation statements)

References 35 publications

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“…Coarctation of the aorta, ventricular septal defect, and pulmonary artery stenosis have been reported as cardiovascular manifestations of the epidermal nevus syndrome [3, 4]. Embryologically, the cardiovascular and skeletal systems are derived from the mesoderm, so the epidermal nevus syndrome would appear to result not only from an ectodermal, but also from a mesodermal defect.…”

Section: Discussionmentioning

confidence: 99%

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Renal Artery Stenosis Associated with Epidermal Nevus Syndrome

Aizawa

Nakamura

Ohyama

et al. 2000

Nephron

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Epidermal nevus syndrome is an unusual neurocutaneous disorder in which epidermal nevi are associated with abnormalities of the skeleton and central nervous system, including the eyes and somtimes the cardiovascular system. We treated a patient in whom the latter included renal artery stenosis. An 18-year-old man with epidermal nevi was diagnosed as having the syndrome based on the additional presence of scoliosis, an arachnoid cyst in the middle cranial fossa, and microphthalmos. Hypertension was diagnosed when the patient was 15 years old. The plasma renin activity (9.7 ng/ml/h) was elevated. Right renal artery stenosis was demonstrated by angiography, and the abdominal aorta was narrowed distal to the ostium of the superior mesenteric artery. The plasma renin activity in the right renal vein (16 ng/ml/h) was higher than contralaterally (10 ng/ml/h). Several cardiovascular manifestations have been reported as a complication of epidermal nevus syndrome. Hypertension in an individual with epidermal nevi and congenital anomalies should prompt a search for a vascular anomaly.

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Section: Discussionmentioning

confidence: 99%

“…Heritability must be ruled out by history. Reported cardiovascular manifestations have not included renal artery stenosis [4]. We recently treated such a patient.…”

Section: Introductionmentioning

confidence: 99%

Renal Artery Stenosis Associated with Epidermal Nevus Syndrome

Aizawa

Nakamura

Ohyama

et al. 2000

Nephron

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“…5a). In a review of 131 cases, Rogers et al [105]found that the head and neck were affected in 42% of cases. Beylot-Barry et al [106]reported on Solomon syndrome with corpus callosum agenesis and myocardiopathy with very impressive linear and reticulated pigmentation of the neck.…”

Section: Nevi With Hamartoma-like Features and True Hamartomasmentioning

confidence: 99%

Genodermatosis with Reticulate, Patchy and Mottled Pigmentation of the Neck – A Clue to Rare Dermatologic Disorders

Itin

Lautenschläger

1998

Dermatology

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Reticulate pigmentation of the neck is a common finding in numerous genodermatoses and acquired diseases. As the neck is readily accessible to medical inspection, it may serve as a diagnostic window for various dermatoses. Several entities out of the spectrum of ectodermal dysplasia present with reticulate or mottled pigmentation on the upper trunk and neck. The most impressive genodermatoses with punctate and reticulate pigmentation affecting the neck are the Naegeli-Franceschetti-Jadassohn syndrome, dermatopathia pigmentosa reticularis and dyskeratosis congenita. Reticulate pigmented anomaly of the flexures and lentiginosis, disorders of cornification and related entities are further genodermatoses which may involve the neck. In our review we have integrated inherited dermatoses which do not obligatorily affect the neck but have been well documented to do so. Our work should give the clinician a checklist on genodermatoses which may produce reticulate and mottled pigmentation on the neck.

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“…Although epidermal nevi are often present at birth, they may develop during childhood and are claimed to be caused by somatic mosaicism, with a breakpoint localized in a number of patients to the long arm of chromosome 1, where the genes of important epithelial structure proteins such as profillagrin, involucrin, loricrin and trichohyalin, as well as the two forms of cellular retinoic acid binding protein are situated [39, 40, 41, 42]. …”

Section: Discussionmentioning

confidence: 99%

Multiple Myofibromas and an Epidermal Verrucous Nevus in a Child with Neurofibromatosis Type 1

et al. 2004

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Neurofibromatosis type 1 (NF1) is a common neurocutaneous autosomal dominant genetic disorder affecting primarily tissues derived from the embryonic neural crest. Two hallmark features of NF1 are the wide range of potentially affected tissues and the enormous phenotypic variability of disease traits even among patients from the same family. We present a boy fulfilling the diagnostic criteria for NF1 with two unusual lesions: infantile myofibromatosis and a verrucous epidermal nevus. To our knowledge this association has never been described before.

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Epidermal nevi and the epidermal nevus syndrome

Cited by 209 publications

References 35 publications

Renal Artery Stenosis Associated with Epidermal Nevus Syndrome

Renal Artery Stenosis Associated with Epidermal Nevus Syndrome

Genodermatosis with Reticulate, Patchy and Mottled Pigmentation of the Neck – A Clue to Rare Dermatologic Disorders

Multiple Myofibromas and an Epidermal Verrucous Nevus in a Child with Neurofibromatosis Type 1

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