neuro cutaneous markers

1. NEUROCUTANEOUS NEUROCUTANEOUS
MARKERSMARKERS
Dr.Ashok.R.Wadhwani

2. 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 .

3. Tuberous sclerosis
Adenoma sebaceum.
Ash leaf spots.
Subungual and gingival fibromas.
Shagreen patches.
Fibrous plaques on the forehead.

4. ADENOMA SEBACEUM:MULTIPLE ANGIFIBROMAS
OF THE FACE .ERYTHEMATOUS PAPULES ON THE
LOWER HALF OF FACE-CHEEKS,NASOLABIAL
FOLDS,SIDES OF THE NOSE AND CHIN.

5. ADENOMA SEBACEUM

6. 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.

7. Ash leaf spots. "confetti" macules. Multiple,
discrete, small, confetti-like, hypopigmented
macules of variable size on the leg.

8. 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.

9. SUBUNGUAL FIBROMA.

10. SUBUNGUAL FIBROMA.

11. GINGIVAL FIBROMA.

12. SHAGREEN PATCH- Leathery plaques of
subepidermal fibrosis,usually situated on the
trunk.

13. SHAGREEN PATCH.

14. VON RECKLING-HAUSEN’S
DISEASE (NF-1)
CAFÉ AU LAIT SPOTS.
AXILLARY FRECKLING.

15. CAFÉ AU LAIT SPOTS. PIGMENTED
MACULES(>1 CM) ON THE TRUNK.
PLEMIFORM NEUROMA SEEN.

16. Several larger (>1.0 cm) cafe-au-lait macules on
the upper chest and multiple small macules in
the axillae (axillary "freckling")

17. STURGE WEBER
SYNDROME
CAPILLARY HAEMANGIOMA.