vitiligo-types pathogenesis clinical features and treatment.

1. VITILIGO
Vishnu narayanan M.R.

2. EPIDERMO-MELANIN
UNIT

3. MELANOGENESIS
tyrosine
↓ tyrosinase
DOPA
↓ tyrosinase
DOPAquinone
↓
↓
indolequinone
↓ polymerization
melanin

4. CONTROL OF
MELANOGENESIS
• Constitutional skin colour
• UV radiation
• Hormones - MSH

5. HYPOPIGMENTATION
HYPOPIGME
NTATION
MELANOPENIC
ANATOMICAL
DEFECTS OF
MELANOCYTES
FUNCTIONAL
DEFECTS OF
MELANOCYTES
NON-
MELANOPENIC
ABNORMALITIES
OF
VASCULATURE

6. MELANOPENIC
HYPOPIGMENTATION
Anatomical
defect
• Vitiligo
• Piebaldism
• Chemicals-
rubber
• Post
inflammatory
Functional defect
• Pityriasis
versicolor
• Endocrine
disorders
• albinism

7. …VITILIGO…
• Vitiligo is an acquired , pigmentary
anomaly of the skin characterised by
depigmented white patches surrounded
by a normal or a hyperpigmented border

8. The Roman physician Celsus first
used the term vitiligo in the second
century AD.
It is interesting to note that the
Rigveda (6000 BC or earlier) named
leukoderma as kilas, meaning a
white spotted deer.

9. ETIOPATHOGENESIS
1.GENETIC:
20 % PATIENTS- POSITIVE FAMILY HISTORY
POLYGENIC INHERITANCE- NALP gene
, HLADR4, CATALASE GENE, …..

10. 2. AUTO-IMMUNE HYPOTHESIS
• Association with other autoimmune disorders-
alopecia areata and thyroid disorders
• Antibodies to melanocytes
• Lymphocytes in early lesions

11. 3. NEUROGENIC HYPOTHESIS
• Segmental vitiligo
• Nerve endings
toxin
destruction of melanocytes

12. Epidemiology
• Incidence – 1 % population
• Race – affects all races
• No sex predisposition
• Age – peak -10-30 years

13. Clinical features
MORPHOLOGY
• Depigmented macules- chalky/ milky white
• Pigment loss- complete/ partial
• Macules- scalloped outline
• Geographical ptterns on fusion of adjacent
lesions
• Hairs – in older lesion- leucotrichia

14. • Trichome vitiligo

15. LEUCOTRICHIA

16. SITES
• Any part can be affected
• Predilection to areas with
repeated friction and trauma
• Dorsal aspect of hands and
feets , elbows , knees

17. PATTERNS
• Vitiligo vulgaris
• Segmental vitiligo
• Generalised vitiligo

18. Vitiligo vulgaris
• Commonest type
• Second decade
• Family history
• Progression – rapid/slow

19. Segmental vitiligo
• Occurs in children
• Not associated with autoimmune disorders
• Depigmentation- dermatomal/
quasidermatomal
• Stable course
• Leucotrichia
• Feathery margin
• Distant lesions- uncommon
• Poor response to treatment

21. Generalised vitiligo
• Extensive lesions
Acrofacial
vitiligo
• Periorificially and
acral parts
• Resistant to
therapy due to
absence of hairs
Lip-tip vitiligo
• lips
• Tip of penis,vulva,
nipples
Vitiligo
universalis
• Widespread
• Multiple
endocrinopathies

22. ACROFACIAL VITILIGO

23. LIP-TIP VITILIGO

24. VITILIGO UNIVERSALIS

25. Course
• Onset < 20 years
• Slowly progressive usually
• Segmental vitiligo – stable
• Spontaneous repigmentation- 10 –
20%
• Acrofacial vitiligo – resistant to
treatment

26.  Absence of melanocyte and melanin in the
epidermis
 e/m confirms the loss of melanocytes which
appears to be replaced by langerhans cells.
 Increased cellularity of the dermis

27. Prognostic factors
• Long standing disease
• Leucotrichia
• Acro facial lesions
• Lesions on resistant
areas
Poor
prognosis

28. Associations
Endocrine disorders
• Diabetes mellitus
• Pernicious anemia
• Addison’s disease
• Hypoparathyroidsm
• Hypothyroidism
• Hyperthyroidism

29. Cutaneous disorders
• Alopecia areata

30. Halo nevus

31. Atopic dermatitis

32. Malignant melanoma

33. Morphea

34. DIAGNOSIS
• Age of onset
• Depigmented macules with
scalloped borders
• Leucotrichia
• Koebner’s phenomenon
• Predeliction to sites of trauma

36. vitiligo albinism
onset Later life At birth
course Depigmentation-
progress/regress
Freckling on
photoexposed parts
Eye involvement Not seen Always present
Response to
treatment
Partial/near complete
response
No response
Differential diagnosis

38. Nevus
achromicus
Vitiligo
onset At birth Not present at
birth
Distribution Segmental/focal Segmental/focal/g
eneralised
Morphology Feathered
margins, uniform
pigment dilution
Scalloped
margins, islands
of pigmentation
Hair No leucotrichia leucotrichia

40. Piebaldism

41. LEUCODERMA
• All depigmented lesions
• Idiopathic- vitiligo
• Chemicals
• Inflammatory skin diseases

43. TREATMENT
DEPENDS ON
• Age of patient
• Extent of disease
• Pattern of disease
• Cosmetic disability
• Effect on quality of life

44. General measures
Physical modality
Medical treatment
Surgical treatment

45. Physical modality
1.Photochemotherapy
 Psoralens + UVA exposure – PUVA
• Mainstay of vitiligo therapy
• Psoralens – tricyclic furocoumarins
8- methoxypsoralen
topical/systemic
• UVA- in special chambers containing UVA
emitting tubes
 PUVA sol – psoralen + sunlight

47. REGIMEN
Topical therapy Alternate days Systemic therapy
Ointment/lotion psoralen (0.6mg/kg)
gradually increasing exposure
till mild erythema
sunlight PUVA – UVA lamps
Protect from sun for 8 hrs
(11:00 - 13:00)
Broad spectrum sunscreens- ZnO

48. RESPONSE TO PHOTOCHEMOTHERAPY
• Repigmentation- slow
• Begins in perifollicular area and periphery of
lesion
• Becomes confluent
• Most readily on face, neck and hairy regions
• Slow responders – acral and non hairy parts

50. • Photo-toxicity
 Excessive exposure to UVA / SUN
 Topical psoralens
 Treatment – withdrawal of psoralen
topical corticosteroids
severe – systemic steroids
• Nausea , vomiting, epigastric
discomfort, giddiness

52. 2. Phototherapy – narrow band UVB (311nm)
• INDICATIONS – in extensive disease>10%
indicated in children and
pregnant women
in patients in whom psoralens C/I
• REGIMEN – Gradually increasing doses of
UVB, given from specialized chambers.
• Safe

53. Medical treatment
• Single lesions esp new
• adjuvantTopical
steroids
• Patient can’t be given
physical modalities
• Rapidly progressive vitiligo
with PUVA
• Vitiligo unresponsive to
psoralens
Systemic
steroids

54. ORAL MINI PULSE
ORAL
STEROIDS-
SINGLE DAY
OR ON 2
CONSECUTIVE
DAYS PER
WEEK

55. OTHERS
• FOR FACIAL LESION
• CALCINEURIN INHIBITOR
TACROLIMUS
,PIMECROLIMUS
• IMMUNOMODULATER
• WEEKLY/FORTNIGHTLY
LEVAMISOLE
• DEPIGMENTING AGENT
MONOBENZYL
ETHER OF
HYDROQUINE
PLACENTAL
EXTRACT
• PHOTOCHEMOTHERAPYKHELLIN

56. SURGICAL TREATMENT
• Indications- sites poorly responsive to
conventional therapy
Patient with stable disease
MELANOCYTE
TRANSFER
BLISTER
GRAFTING
PUNCH
GRAFTING
SPLIT
THICKNESS
SKIN
GRAFTING

57. Treatment guidelines
LOCALISED DISEASE
New lesions Topical steroids
Old lesions Topical PUVA/ PUVA sol
EXTENSIVE DISEASE
New lesions Oral steroids + PUVA/PUVA sol
NBUVB
Rapid increase Oral steroids + PUVA/PUVA sol
/NBUVB
Old lesions Oral PUVA/PUVA sol/NBUVB
Intolerence to PUVA/NBUVB Oral steroids
GENERALISED LESIONS Monobenzyl ether of hydroquinone