VITILIGO

- CLINICAL CLASSIFICATION -

George-Sorin Ţiplica

Colentina Clinical Hospital

Bucharest, Romania

Gregor Johann Mendel

1822 – 1884

Czech-German Augustinian

monk and scientist

Studied the inheritance of

certain traits in pea plants

Founder of genetics

Why classifications are useful?

Prognosis evaluation

Treatment options

Clinical studies, meta-analysis

Reimbursment, DRG system

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

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

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

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.

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.

Clinical classification

FOCAL Most common distribution: -trigemminal nerve -neck -trunck

GENERALIZED - most common pattern - symmetrical 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

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

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.

Clinical classification

SEGMENTAL

- does not cross the middle line -usually in children

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

MIXED FORM

– combines segmental,

acrofacial and/or

generalized distribution

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.

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

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

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

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

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

VIDA score Vitiligo Disease Activity

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

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 repigmentation

1 year or more -1

www.dermabest.com/Vitiligo_Disease_Activity_score

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)

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

Thank you!