Epidermal nevus
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This document discusses epidermal naevi, which are congenital developmental defects or birthmarks of the skin and mucosa. It describes different types of epidermal naevi classified based on the level of defect (epidermal, dermal, subcutaneous) and component cell (vascular, connective tissue, melanocytic). Verrucous epidermal naevi, also known as verrucous nevus or nevus verrucosus, are discussed in detail. They are keratinocyte hamartomas that can be either epidermolytic or non-epidermolytic types, with the latter having greater malignant potential and possible associations with extracutaneous abnormalities. Clinical features
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Epidermal nevus
- 2. Introduction • Latin : ‘maternal impression’ / ‘birthmark’ • Developmental Defect • Cutaenous Hamartoma • Well-circumscribed, fixed • non-neoplastic skin / mucosal lesion • usually present at or soon after birth • qualified according to origin
- 3. Aetiology • Environmental – Intrauterine Infections – Ionizing Radiation – Teratogenic Drugs – Alcohol / Smoking – Nutritional Deficiencies – Maternal Disease – Parental Exposure • Genetic – Microdeletion – Chromosomal Syndromes – Mutations – Genetic Mosaicism
- 4. Classification • Based on level of defect Epidermal / Dermal / Subcutaneous • Based on Component Cell Vascular / Connective Tissue / Melanocytic • Congenital / Acquired
- 5. Classification : Epidermal Keratinocyte naevi • Verrucous epidermal naevus (VEN) – Epidermolytic VEN – Non-epidermolytic VEN Sebaceous naevi • Naevus sebaceus Follicular naevi • True hair-follicle naevus • Comedo naevus • ‘Acne-free’ naevus • Basaloid follicular hamartoma • Dilated pore naevus • Hairy malformation of the palms and soles
- 6. Apocrine naevi • True apocrine naevus • Syringocystadenoma papilliferum Eccrine naevi • True eccrine naevus • Eccrine angiomatous hamartoma • Porokeratotic eccrine ostial and dermal duct naevus Becker’s naevus Inflammatory epidermal naevi • CHILD naevus • ILVEN Other naevoid epidermal disorders • Naevoid psoriasis • Linear lichen planus • Darier-like epidermal naevus • Hailey–Hailey-like epidermal naevus • Linear porokeratosis • Atrophoderma of Moulin • ‘Blaschkitis’ Epidermal naevus syndrome
- 7. Classification : Subepidermal Connective tissue naevi Collagen naevi • Familial cutaneous collagenoma • Eruptive collagenoma • Shagreen patch • Other collagenomas Elastic naevi • Perforating elastoma • Juvenile elastoma • Buschke–Ollendorff syndrome • Papular elastorrhexis • Naevus anelasticans • Other elastomas
- 8. Proteoglycan naevi • Mucinous naevus • Fibrous hamartoma of infancy Muscle naevi • Infantile myofibroma • Congenital smooth muscle hamartoma • Diffuse smooth muscle hamartoma • Congenital leiomyoma • Striated muscle naevi Fat naevi • Naevi lipomatodes cutaneous superficialis • Lipoblastoma • Encephalocraniocutaneous lipomatosis • Congenital lipomatosis • Neurolipomatosis • ‘Michelin tyre’ baby
- 9. Keratinocyte Nevi Verrucous epidermal naevi Syn : Nevus Verrucosus / Nevus Unius Lateris • Cutaneous hamartomas comprising keratinocytes • Congenital • non- inflammatory • Prevalence 0.1–0.5% • M:F = 1:1 • Epidermolytic / Non-Epidermolytic types
- 10. Epidermolytic VEN • Mutations on genes KRT1 / KRT10 • Associated with BIE • Insignificant Malignant Potential • No association with extracutaneous abnormalities keratin genes are expressed only in epithelia
- 11. Clinical Features • Slightly pigmented brown velvety or warty streaks or plaques • Hyperpigmented and more warty with age • Flexural lesions macerated and foul smelling • Rarely :- blisters at birth verrucous with age • Striate palmoplantar keratoderma / Ainhum reported in a child with extensive systematized VEN
- 13. HPE Hyperkeratosis Acanthosis Hypergranulosis Inc Keratohyaline Granules perinuclear vacuolization of keratinocytes s/o EHK Few inflamm cells
- 14. Non-Epidermolytic VEN • Mosaic Chr 06 Trisomy • FGFR3 Mutation • Assoc with PIC3CA and PTEN mutations • Significant Malignant Potential > 40 yrs • Associated with extracutaneous manifestations
- 15. Clinical Features • present at birth / childhood / 55 yrs of age • Crusted, hyperkeratotic plaques on the head and upper trunk • Birth white macerated appearance pink / slightly pigmented, velvety streaks or plaques darken and the surface becomes more warty • Flexural lesions macerated and foul smelling
- 16. • aa
- 17. Associations : • Nail fold Paronychia / ridging / splitting / discoloration or dystrophy • Wooly-Hair • Megalopinna • Aplasia Cutis • Epidermal Nevus Syndrome • Proteus Syndrome
- 18. HPE • Hyperkeratosis ; Columns of Parakeratosis • Acanthosis, Papillomatosis, Focal hypergranulosis • 10% of lesions show a distinctive ‘church-spire’ pattern of acanthosis and hyperkeratosis, resembling acrokeratosis verruciformis • 5% show features resembling seborrhoeic keratoses, that is hyperkeratosis, papillomatosis, acanthosis and horn pseudocysts • Immature adnexae (hair follicles, sebaceous, eccrine or apocrine glands)
- 19. Treatment Topical applications • SA / LA / RA preparations Decrease Hyperkeratosis • Podophyllum • 5-fluorouracil • calcipotriol and calcitriol Systemic retinoid therapy. Isotretinoin / etretinate / Acitretin can reduction of hyperkeratosis (in epidermolytic lesions) Dermabrasion
- 20. Lasers overcomes problems such as hypertrophic scarring, pigmentary changes and partial recurrence Argon laser is helpful for softer, less hyperkeratotic lesions Continuous-wave CO2 laser vaporization Extensive VEN Pulsed CO2 thinner and softer VEN Er:YAG lasers with greater coagulative capacity – Lower recurrence Pulsed Ruby Laser Surgery Multi-modal, Multi-stage Dermis removal only effective
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