The document provides a comprehensive overview of vitiligo, including its clinical classification, subtypes (non-segmental and segmental), and associated diseases. It discusses the challenges in defining and assessing vitiligo, the absence of consensus in classification systems, and various methods of assessing disease activity. It emphasizes that predicting future evolution and localization remains difficult due to the complexity and variability of vitiligo.

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!