Dr. George Sorin Tiplica Presentation from the World Vitiligo Symposium 2011. Sponsored by the VR Foundation.

1. VITILIGO
- CLINICAL CLASSIFICATION -
George-Sorin Ţiplica
Colentina Clinical Hospital
Bucharest, Romania

2. Gregor Johann Mendel
 1822 – 1884
 Czech-German Augustinian
monk and scientist
 Studied the inheritance of
certain traits in pea plants
 Founder of genetics

3. Why classifications are useful?
 Prognosis evaluation
 Treatment options
 Clinical studies, meta-analysis
 Reimbursment, DRG system

4. Classification: general considerations
 Classification system A
 Classification system B
 Classification system C
 Classification system D
Validation by
 Patho-mechanism
 Treatment
 Evolution / prognosis
Classification system B

5. Clinical classification
 What can be observed?
 Objective criteria
 Disease distribution
 Speed of spreading
 Lesion aspect (shape, dimension, color)
 Subjective criteria
 Symptoms
 Quality of life
 Who can observe?
 General practitioner / dermatologist
 Patient
 Computer

6. Clinical classification: vitiligo
 There is a current lack of consensus in definition and assessment
methods which makes difficult any classification
 Initial site involvement does not predict future evolution,
localisation and activity of the disease
 Many attempts to classify the disease
 Several subtypes of vitiligo
 Based on the distribution of lesions

7. Vitiligo - classification
 Non-segmental vitiligo (type A)
 most common subtype
 widespread macules often symmetrically placed
 frequently involves acral areas, skin surrounding body
orifices and extensor surfaces
 Segmental vitiligo (type B)
 dermatomal or blaschkolinear distribution
Koga M. Vitiligo: a new classification and therapy. Br J Dermatol. 1977 Sep;97(3):255-61.

8. Non-segmental vitiligo
 Universal vitiligo (almost complete
depigmentation of the cutaneous surface)
 Generalized vitiligo (most frequent form)
 Acrofacial vitiligo (limited to acral and areas
surrounding the orifices)
 Mucosal vitiligo (limited to mucosa)
 Focal (depigmented macules located in an
isolated area without a dermatomal distribution)
 Koebner phenomenon present
 Characteristics
 usually slowly progressive
 spontaneous repigmentation in 10-20% of patients
Handa S, Kaur I. Vitiligo: clinical findings in 1436 patients. J Dermatol 1999; 26:653.

9. Clinical classification
FOCAL GENERALIZED
- most common pattern
Most common distribution:
- symmetrical distribution
-trigemminal nerve
-neck
-trunck
Bologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920
Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621

11. Associated diseases
 Autoimmune origin:
 Thyroid disease (hyperthyroidism, hypothyroidism)
 Addison’s disease
 Pernicious anemia
 Alopecia aerata
 Diabetes mellitus
 Myasthenia gravis
 Halo nevus
 Malignant melanoma
Bologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920
Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621
Burns T, Breathnach S, Cox N, Griffiths C. Rook’s Textbook of Dermatology, 8th Ed, 2010, vol 3, p 58.46-58.49

12. Segmental vitiligo
 A less common subtype
 Unilateral depigmented macules and patches that
completely or partially occur in a dermatomal or
blaschkolinear distribution
 Characteristics
 earlier age of onset
 spreads rapidly after initial appearance
 less commonly associated with other autoimmune
diseases
 pathogenesis: a neurogenic sympathetic abnormality or
a disorder of cutaneous mosaicism
Taïeb A, Picardo M. Clinical practice. Vitiligo. N Engl J Med 2009; 360:160.
Hann SK, Lee HJ. Segmental vitiligo: clinical findings in 208 patients. J Am Acad Dermatol 1996; 35:671.
Mazereeuw-Hautier J, Bezio S, Mahe E, et al. Segmental… J Am Acad Dermatol 2010; 62:945.
Halder, RM, Taliaferro, SJ. Vitiligo. In: Fitzpatrick's Dermatology in General Medicine, 7th ed,
Wolff, K, Goldsmith LA, Katz, SI, et al (Eds), McGraw Hill 2008.

13. Clinical classification
MIXED FORM
SEGMENTAL – combines segmental,
- does not cross the middle line acrofacial and/or
-usually in children generalized distribution
Bologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920
Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621

15. Facial segmental vitiligo
 Not always correspond to dermatomal
distribution
 Facial SV classification (based on morphological
similarities in numerous clinical observations)
 Type I-a:mid-level face from the forehead to the lower
cheek (28,8%)
 Type I-b: forehead and scalp hair
 Type II: lower face and the neck area (16%)
 Type III: lower face and the neck area (14,4%)
 Type IV: mid-level face from the forehead to the lower
cheek, but selectively appeared on the right side of the
face and did not cross the midline
 Type V: lesions were distributed mostly around the right
orbital area
Kim, D.-Y., Oh, S. H., Hann, S.-K. Classification of segmental vitiligo on the face: clues for prognosis.
British Journal of Dermatology; May2011, Vol. 164 Issue 5, p1004-1009.

17. Clinical variants of vitiligo
 Trichrome vitiligo
 Both depigmented and hypopigmented macules
 Hypopigmented macules become depigmented over time
 Qvadrichrome vitiligo
 Also associates marginal and perifollicular hyperpigmentation
 Darker skin types
 More often in areas of repigmentation
 Pentachrome vitiligo (vary rare)
 Blue-gray hyperpigmentation (dermal melanine incontinence, areas affected by
postinflammatory hyperpigmentation in which vitiligo develops)
 Confetti type (vitiligo ponctue)
 Very numerous small, discrete hypopigmented macules
 Inflammatory vitiligo
 Erythema of the margins
Bologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920
Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621

18. Rare syndromes
 Vogt-Koyanagi-Harada Syndrome
 Vitiligo and uveitis, aseptic meningitis, dysacusis, tinnitus,
poliosis, alopecia
 T-cell mediated autoimmune disease
 Associated with other autoimmune disorders
 Alezzandrini Syndrome
 Facial vitiligo and poliosis, deafness, unilateral retinal
degeneration
Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621

19. Vitiligo in children
 very rarely at birth
 descending order of frequency:
 generalized
 focal
 segmental
 acrofacial
 mucosal
 universal
 Halo nevus present – search for vitiligo
Paller A, Mancini A. Hurwitz Clinical Pediatric dermatology, 3rd Ed, 2006, p 226-229

21. Differential Diagnosis
 According to site
 Face: tinea, pityriasis versicolor, pityriasis alba, post-
inflammatory hypopigmentation, chemical leucoderma,
sarcoidosis, piebaldism, hypopigmentation after cosmetic
procedures
 Hands: chemical leucoderma, scleroderma
 Trunk: scleroderma, pityriasis versicolor, tuberous sclerosis
 Anogenital: lichen sclerosus, lichen atrophicus
Bologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920
Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621
Burns T, Breathnach S, Cox N, Griffiths C. Rook’s Textbook of Dermatology, 8th Ed, 2010, vol 3, p 58.46-58.49

22. Vitiligo: methods of assessment
 Vitiligo European Task Force: a system
(derived from SCORAD) which combines
 analysis of extent: the rule of 9
 stage of disease (staging): the disease is
staged 0–3 on the largest macule in each body
region
 disease progression (spreading): based on
Wood’s lamp examination
Taıeb A, Picardo M on behalf of the VETF members. The definition and assessment of vitiligo:
a consensus report of the Vitiligo European Task Force. Pigment Cell Res. 2007, 20; 27–35

23. VIDA score
 Vitiligo Disease Activity
Vitiligo Activity Time Period VIDA Score
Active Under 6 weeks +4
Active 6 weeks - 3 months +3
Active 3 - 6 months +2
Active 6 - 12 months +1
Stable 1 year or more 0
Stable with spontaneous
1 year or more -1
repigmentation
 Based on the patient's own opinion of the present disease activity over time
 Active Vitiligo refers to either one of the following:
 Expansion of existing lesions
 Appearance of new lesions.
 Asses response to treatment
www.dermabest.com/Vitiligo_Disease_Activity_score

24. VASI
 Introduced by Hamzavi et al. in 2004
 A quantitative parametric score
 Derived from the PASI (psoriasis area and severity
index) score widely used for psoriasis (Fredriksson
and Pettersson, 1978)
 VASI regions
 hands, upper extremities (excluding hands), trunk, lower
extremities (excluding the feet) and feet
 the face and neck areas are assessed separately
 VASI = S (all body sites)(hand units)·(depigmentation)

25. Conclusions
 At the moment, impossible to predict future
evolution, localisation and activity of the disease
 Lack of consensus vitiligo classification and
assessment methods
 Based on pathogenesis
 Non-segmental vitiligo
 Segmental vitiligo

26. Thank you!