ACROFACIAL VITILIGO
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ACROFACIAL VITILIGO DR SASI ATTILI - Disclaimer- This PPT is loaded as student material "as is", from the VRF Vitiligo Master Class Barcelona November 2011; VRF does not endorse or otherwise approve it.
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ACROFACIAL VITILIGO
- 1. Sasi.K. Attili (UK), V R Attili (India)
- 2. - Disclaimer- aterial "as is", from the VRF Vitiligo Master Class Barcelona November 2011; VRF does no
- 4. • Is mucosal vitiligo part of acrofacial? • What about anogenital lesions?
- 5. 1. Acrofacial/ Lip-tip vitiligo currently considered as a limited form of generalized vitiligo (NSV) because of symmetrical acral involvement with facial lesions. 2. Vitiligoid lichen sclerosus (VLS)
- 6. Lichen Sclerosus et Atrophicus, Hemorrhagic and Vitiligoid Type. Julio M. Borda, Luis M. Mirande, and Jorge Abulafia. Abstracted by Orlando Canizares for Arch Dermatol. 1961; 84: 698-706 Borda JM, Balas Re. Lichen sclerosus et atrophicus of a vitiligoid type (In Spanish). Arch Argent Dermatol 1961; 11: 419–424. Borda JM, Abulafia J, Jaimovich L. Syndrome of circumscribed scleroatrophies. Dermatol Iber Lat Am 1968; 3: 179–202. (In English) Attili VR, Attili SK: Lichenoid inflammation in vitiligo: A clinical and histopathologic review of 210 cases. Int J Dermatol 47:663-669, 2008 Attili VR, Attili SK. Vitiligoid lichen sclerosus: a reappraisal. Indian J.Dermatol. Venereol. Leprol. 2008; 74(2):118-21. Attili VR, Attili SK. Lichen sclerosus of lips: a clinical and histopathologic study of 27 cases. International journal of dermatology. 2010; 49(5):520-6.
- 7. 1. Acrofacial/ Lip-tip vitiligo currently considered as a limited form of generalized vitiligo (NSV) because of symmetrical acral involvement with facial lesions. 2. Vitiligoid lichen sclerosus (VLS) superficial variant of lichen sclerosus- presents as asymptomatic vitiligoid depigmentation involving acral, facial and genital areas i.e clinical= vitiligo, histology= LS
- 8. Since VLS and acrofacial vitiligo look alike and both involve the same areas, can they both be differentiated?
- 9. Attili VR, Attili SK: Lichenoid inflammation in vitiligo: A clinical and histopathologic review of 210 cases. IJD 2008
- 11. • Aim: characterisation and differentiation of acrofacial vitiligoid lesions • History and a thorough clinical examination in all patients • A single biopsy was taken from patients with typical macular lesions. Multiple biopsies were taken when ever possible from patients with atypical lesions
- 12. • Acrofacial vitiligoid lesions were observed on clinical examination in 54 patients. • Genital depigmentation- associated in 26/54 patients.
- 13. Source & Number of LS* VLS** VL*** Biopsies reviewed Acral (33/54) 7 8 (24%) 18 Facial (29/54) 2 17 (59%) 10 Genital (17/26) 2 13 (76%) 2 LS*- Both clinical and histological features of LS VLS**- Clinical vitiligo with histological features of LS VL***- No clinical or histological features of LS= vitiligo
- 14. Pattern Clinical LS Histological LS Potential missed diag. Acral-Facial (28) 1(4%) 18/28 patients (64%) 10 / 28 (36%) Acral-Facial-Genital (26) 10(38%) 23/26 patients (88%) 3 / 26 (12%) 6/26 patients (AFG) had biopsies from all three areas and 4 had LS in all the three areas. AF and AFG depigmentation therefore probably belong to the same spectrum with AFG being the complete expression of the disease
- 21. • Racially restricted phenomenon? • Casual clinical diagnosis of vitiligo. Biopsies are not taken from atypical vitiligo lesions Genital area is not routinely examined and patients • don’t complain re: genital lesions. Most genital lesions in this series were discovered on direct questioning and persuasive inspection
- 22. • Initial objective of differentiating AF vitiligo from VLS was only successful to a limited extent • Though individual lesions of vitiligo and VLS can be differentiated in most cases by histo review • a significant number of cases that do not fall into either category • Features of both vitiligo (symmetrical acral depigmentation) and LS (clinical and histological features) were observed concurrently in some patients.
- 23. 1. Lesions of vitiligo may have histologic changes typical of LS (VLS) 2. Lesions typical of vitiligo both clinically and histologically, are found in conjunction with LS
- 24. True association of vitiligo and LS? • Association is not seen with the other clinical types of vitiligo • The clinical lesions are also atypical with punctate & guttate depigmentation in acral distribution Acrofacial vitiligoid lesions are early/ abortive lesions of LS? • Bilateral symmetry over hands and feet is not a known feature of LS
- 25. • Further focused studies are needed to find out: If this is a true association, superimposition of one over the other or a distinct disease different from both vitiligo and LS. • Mucosal, genital and acrofacial vitiligo are part of the same spectrum • Acrofacial vitiligo is probably distinct from Generalized vitiligo with acral lesions • shows punctate/ guttate lesions spreading in a centripetal pattern, rarely affects the trunk and shows a high prevalence/ predisposition to LS • ? Explains poor treatment response in acral lesions
Editor's Notes
- GM. Thank you… I am Sasi Atttili and am a Dermatologist working in the UK. Over the last7 years I have been closely involved in an ongoing study on vitiligoid lesions along with my Dad who is a Dermatologist in India and I would like to share our experience of 54 very interesting cases of AF vitiligo. which to us suggests an etiology different from generalised NSV. We are presenting 54 cases of vitiligo with essentially Acrofacial and genital involvement. The clinical and histological features show a pattern that appears to be distinct from vitiligo vulgaris.
- What is acrofacial vitiligoIn a good number of these cases, the genital area is also affected. These cases are currently classified as acrofacial/Lip-tip/Mucosal vitiligo. We believe ‘Acral vitiligo’ is more appropriate because the distinctive feature is that the trunk is mostly spared and progression pattern is centripetal.
- Acral lesions seen in Vitiligo vulgaris appear to be different from exclusive Acral vitiligo, variously called as acrofacial, Lip-tip and mucosal vitiligo.
- Current status- unclear. In NSV and diff from mucosal vitiligo
- AF depigmentation is currently considered as a limited form of generalized vitiligo. In this regard, it is interesting to note that VLS, a recent rediscovery also affects acrofacial and genital areas. A biopsy can differentiate the two diseases.
- LSA of vitiligoid type was first reported from Argentina by Prof. Borda et al in 3 publications but did not attract global attention and follow up. Rediscovery of VLS was an off shoot of our histopathological study of vitiligo which indicated the microinflammatory nature of the pathogenesis. During the course of this study, we have discovered one other microinflammatory disease resulting in depigmentation different from vitiligo because it had typical histological features of LS. These vitiligoid lesions were mostly seen among patients with oral/genital and acral depigmentation.
- AF depigmentation is currently considered as a limited form of generalized vitiligo. In this regard, it is interesting to note that VLS, a recent rediscovery also affects acrofacial and genital areas. A biopsy can differentiate the two diseases.
- Since VLS and AF vitiligo involve the same areas and look alike on clinical examination, differentiation of the two diseasescan be made only by histopathological review. Since histological information on vitiligo and VLS is recent, we need to refresh this knowledge.
- Vitiligo histopathology has long been considered non-specific but, we now believe there is sufficient evidence to say that it is a microinflammatory disease of the nature of a sub-clinical lichenoid dermatitis with its known sequel of atrophy / sclerosis.
- In few cases, scanty cellular infiltration at the interface resolving with insignificant dermal papillary homogenization has been observed. We believe these lesions are milder abortive forms of VLS. How ever, since these are not typical features of LS, evolving vitiligo can not be excluded beyond doubt.
- Having considered the histological differentiation of VLS and vitiligo, we have reviewed clinical and histological features in 54 cases of acrofacial depigmentation.
- Not all cases end up with clear LS features. Here the AFG depigmentation clinical pattern is similar to other cases, but firm clinical/ histological evidence of LS is lacking.