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Acrofacial vitiligo has features of both vitiligo and lichen sclerosus. An association or a distinct pattern disease?: A clinical and histopathological review of 54 cases Sasi.K. Attili (UK) , V R Attili (India)

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 has features of both vitiligo and lichen sclerosus. An association or a distinct pattern disease?:

A clinical and histopathological review of 54 cases

Sasi.K. Attili (UK), V R Attili (India)

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What is acrofacial vitiligo?

- 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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Vitiligo vulgaris with acral lesions

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Current status of acrofacial vitiligo

• Is mucosal vitiligo part of acrofacial?

• What about anogenital lesions?

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Acrofacial depigmentation:differential

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)

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Vitiligoid Lichen sclerosus

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.

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Acrofacial depigmentation: 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

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Since VLS and acrofacial vitiligo look alike and both involve the same areas, can they

both be differentiated?

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Histopathological evolution of vitiligo:Inflammatory, stable and late lesions

Attili VR, Attili SK: Lichenoid inflammation in vitiligo: A clinical and histopathologic review of 210 cases. IJD 2008

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VLS or Vitiligo?

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A review of acrofacial vitiligoid lesions (2003-2010)

• 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

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Results

• Acrofacial vitiligoid lesions were observed on clinical examination in 54 patients.

• Genital depigmentation- associated in 26/54 patients.

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Prevalence of LS/ VLS / Vitiligoid lesions(Vitiligo)

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

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Clinical and histological features in Acrofacial (28) and acrofaciogenital (26) depigmentation

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

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In 30/ 54 cases, acral lesions were punctate and guttate merging in to larger macular depigmented

areas

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AFG-2 -Atrophic depigmentation in all three areas

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AFG-5

Vitiligo?

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AFG-6 Punctate acral lesions and leucotrichia= Vitiligo?

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Acrofacial depigmentation- 1. No genital lesion.Slightly thickened lip lesion: LS stage-3

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Acrofacial depigmentation -2. Thickened milk white patches over lower lip-Progressive LS stage.

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How did these cases escape notice? Why hasn’t this been reported earlier?

• 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

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Conclusions

• 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.

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Lichen Sclerosus et Atrophicus- Vitiligoid type. (1960-68 Three publications in Spanish)

Julio M. Borda, Luis M. Mirande, and Jorge Abulafia.

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

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Can we correlate the clinical/ histological pattern?

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

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Conclusions:

• 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

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THANK YOU

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