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 distributionKoga 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 patientsHanda S, Kaur I. Vitiligo: clinical findings in 1436 patients. J Dermatol 1999; 26:653.
Clinical classification FOCAL GENERALIZED - most common patternMost 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
Associated diseases Autoimmune origin: Thyroid disease (hyperthyroidism, hypothyroidism) Addison’s disease Pernicious anemia Alopecia aerata Diabetes mellitus Myasthenia gravis Halo nevus Malignant melanomaBologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621Burns 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 mosaicismTaïeb 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: Fitzpatricks Dermatology in General Medicine, 7th ed,Wolff, K, Goldsmith LA, Katz, SI, et al (Eds), McGraw Hill 2008.
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
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 areaKim, 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 marginsBologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920Fitzpatrick’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 degenerationFitzpatrick’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 vitiligoPaller 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 atrophicusBologna JL, Jorizzo J, Rapini R. Dermatology, 2nd Ed, 2008, p 913-920Fitzpatrick’s Dermatology in Internal Medicine, 7th Ed. 2008, vol 2, p 616-621Burns 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 examinationTaıeb 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 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 patients 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 treatmentwww.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