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.TRANSCRIPT
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)
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.
Vitiligo vulgaris with acral lesions
Current status of acrofacial vitiligo
• Is mucosal vitiligo part of acrofacial?
• What about anogenital lesions?
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)
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.
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
Since VLS and acrofacial vitiligo look alike and both involve the same areas, can they
both be differentiated?
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
VLS or Vitiligo?
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
Results
• Acrofacial vitiligoid lesions were observed on clinical examination in 54 patients.
• Genital depigmentation- associated in 26/54 patients.
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
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
In 30/ 54 cases, acral lesions were punctate and guttate merging in to larger macular depigmented
areas
AFG-2 -Atrophic depigmentation in all three areas
AFG-5
Vitiligo?
AFG-6 Punctate acral lesions and leucotrichia= Vitiligo?
Acrofacial depigmentation- 1. No genital lesion.Slightly thickened lip lesion: LS stage-3
Acrofacial depigmentation -2. Thickened milk white patches over lower lip-Progressive LS stage.
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
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.
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
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
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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