Post on 18-Dec-2014
description
NEUROCUTANEOUS NEUROCUTANEOUS MARKERSMARKERS
Dr.Ashok.R.Wadhwani
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 .
Tuberous sclerosis
Adenoma sebaceum.Ash leaf spots. Subungual and gingival fibromas.Shagreen patches.Fibrous plaques on the forehead.
ADENOMA SEBACEUM:MULTIPLE ANGIFIBROMAS OF THE FACE .ERYTHEMATOUS PAPULES ON THE
LOWER HALF OF FACE-CHEEKS,NASOLABIAL FOLDS,SIDES OF THE NOSE AND CHIN.
ADENOMA SEBACEUM
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.
Ash leaf spots. "confetti" macules. Multiple, discrete, small, confetti-like, hypopigmented macules of variable size on the leg.
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.
SUBUNGUAL FIBROMA.
SUBUNGUAL FIBROMA.
GINGIVAL FIBROMA.
SHAGREEN PATCH- Leathery plaques of subepidermal fibrosis,usually situated on the trunk.
SHAGREEN PATCH.
VON RECKLING-HAUSEN’S DISEASE (NF-1)
CAFÉ AU LAIT SPOTS. AXILLARY FRECKLING.
CAFÉ AU LAIT SPOTS. PIGMENTED MACULES(>1 CM) ON THE TRUNK.
PLEMIFORM NEUROMA SEEN.
Several larger (1.0 cm) cafe-au-lait macules on the upper chest and multiple small macules in the axillae (axillary "freckling")
STURGE WEBER SYNDROME
CAPILLARY HAEMANGIOMA.