neuro cutaneous markers
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NEUROCUTANEOUS NEUROCUTANEOUS MARKERSMARKERS
Dr.Ashok.R.Wadhwani
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NEUROCUTANEOUS SYNDROMES
This is a group of genetic disorders, also known as the phakomatoses,which produces a variety of developmental abnormalities that frequently involve skin, along with an increased risk of nervous system tumors. These disorders are inherited as autosomal dominant conditions .
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Tuberous sclerosis
Adenoma sebaceum.Ash leaf spots. Subungual and gingival fibromas.Shagreen patches.Fibrous plaques on the forehead.
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ADENOMA SEBACEUM:MULTIPLE ANGIFIBROMAS OF THE FACE .ERYTHEMATOUS PAPULES ON THE
LOWER HALF OF FACE-CHEEKS,NASOLABIAL FOLDS,SIDES OF THE NOSE AND CHIN.
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ADENOMA SEBACEUM
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ASH LEAF SPOTS. Hypopigmented macules. The average size is 1 to 3 cm, and the common shapes are oval,
polygonal, and lance-ovate, whereas the less common shapes are dermatomal and confettilike.
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Ash leaf spots. "confetti" macules. Multiple, discrete, small, confetti-like, hypopigmented macules of variable size on the leg.
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Detection of ash leaf spots may require Wood's lamp examination(UVA), especially in fair-skinned individuals. An ash leaf spot on the scalp will result in poliosis, which is a circumscribed patch of gray-white hair.
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SUBUNGUAL FIBROMA.
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SUBUNGUAL FIBROMA.
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GINGIVAL FIBROMA.
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SHAGREEN PATCH- Leathery plaques of subepidermal fibrosis,usually situated on the trunk.
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SHAGREEN PATCH.
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VON RECKLING-HAUSEN’S DISEASE (NF-1)
CAFÉ AU LAIT SPOTS. AXILLARY FRECKLING.
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CAFÉ AU LAIT SPOTS. PIGMENTED MACULES(>1 CM) ON THE TRUNK.
PLEMIFORM NEUROMA SEEN.
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Several larger (1.0 cm) cafe-au-lait macules on the upper chest and multiple small macules in the axillae (axillary "freckling")
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STURGE WEBER SYNDROME
CAPILLARY HAEMANGIOMA.
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