Home About us Editorial board Ahead of print Current issue Archives Instructions Submit article Search Subscribe Contacts Login
  • Users Online: 597
  • Home
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 55-58

The convergence of extragenital lichen sclerosus with generalized vitiligo – an unusual presentation


1 Department of Dermatology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Pathology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission11-Feb-2020
Date of Decision11-Apr-2020
Date of Acceptance27-Jul-2020
Date of Web Publication07-Apr-2021

Correspondence Address:
MD Aditi Dhanta
Department of Dermatology, Venereology and Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/Pigmentinternational.Pigmentinternational_

Rights and Permissions
  Abstract 


Lichen sclerosus (LS), also known as Csillag’s disease, is an autoimmune, chronic inflammatory dermatosis with predilection for localized involvement of anogenital skin. However, extragenital LS is not uncommon. There is strong association of autoimmune disorders with LS. Vitiligo is an autoimmune disorder characterized by progressive loss of melanocytes causing appearance of localized and/or generalized depigmentation of the skin and/or mucous membranes. There have been a few reported cases in the literature of the coexistence of acrofacial vitiligo and LS. Here, we report a case of 70 year old female with coexistence of extragenital lichen sclerosus and generalised vitiligo. Interestingly in our patient LS lesions developed over generalised vitiligo patches. Histopathology confirmed the coexistence of vitiligo and LS.

Keywords: Lichen sclerosus (LS), vitiligo, vitiligoid LS


How to cite this article:
Arora K, Dhanta A, Hazarika N, Joshi P. The convergence of extragenital lichen sclerosus with generalized vitiligo – an unusual presentation. Pigment Int 2021;8:55-8

How to cite this URL:
Arora K, Dhanta A, Hazarika N, Joshi P. The convergence of extragenital lichen sclerosus with generalized vitiligo – an unusual presentation. Pigment Int [serial online] 2021 [cited 2021 Apr 14];8:55-8. Available from: https://www.pigmentinternational.com/text.asp?2021/8/1/55/313139




  Introduction Top


Lichen sclerosus (LS) is a chronic inflammatory dermatosis characterized by small, porcelain white, sclerotic areas distributed predominantly on anogenital region. Several lines of evidence support the hypothesis of an autoimmune etiology such as the presence of circulating antibodies targeting extracellular matrix protein 1 and frequent association with autoimmune disorders.[1] Vitiligo, an autoimmune disorder characterized by well-defined macules occur secondary to melanocyte dysfunction or loss is a well-recognised entity. Lesions having clinical appearance of vitiligo and histological features of LS were considered as ’vitiligoid LS’, a superficial variant proposed by J. M. Borda in 1968.[2] There have been a few reported cases in the literature of the coexistence of acrofacial vitiligo and LS.


  Case Report Top


A 70 yr old female presented with chief complains of multiple depigmented lesions over the entire trunk, extremities and genitalia for past 2 years. Then 6 months later, she started developing few itchy lesions over previous lesions on forearms and shoulders. On cutaneous examination, there were multiple discrete well defined depigmented macules of varying size 0.5 × 0.5 cm to 4 × 3 cm over trunk, bilateral upper extremities and genitalia [Figure 1]a. Over the depigmented macules on forearms and shoulders, there were multiple erythematous papules and plaques of size 0.5 × 0.5 cm to 1.5 × 1.5 cm with fine scaling and atrophy [Figure 1]b-1c. Dermoscopy from depigmented macules using DermLite DL200 Hybrid-3Gen; polarized mode, × 10 magnification showed sharp border with absent pigment network within the macule and from sclerotic lesions showed follicular plugging with white clouds and fine whitish scales over the background of erythematous halo were suggestive of vitiligo and LS respectively [Figure 2].
Figure 1 Depigmented macules over vulva with no atrophy, (b) multiple erythematous papules and plaque with atrophy and scaling over depigmented macule on shoulder, (c) multiple erythematous papules and plaques with atrophy and scaling over depigmented macules on forearm

Click here to view
Figure 2 Follicular plugging with white clouds and fine whitish scales over the background of erythematous halo seen on dermoscopy from sclerotic lesions using DermLite DL200 Hybrid − 3Gen; polarized mode, ×10 magnification

Click here to view


Skin biopsy were performed from both the lesions. There was decreased melanin in basal layer on biopsy from depigmented macule over forearm suggestive of vitiligo. Biopsy from hypopigmented sclerotic plaque over shoulder revealed follicular plugging, epidermal atrophy with lichenoid infiltrate and dermal hyalinisation in papillary dermis, features consistent with Lichen sclerosus [Figure 3]a, 3b.
Figure 3 (a) Follicular plugging with lichenoid inflammation in papillary dermis (4x, H&E), b) lichenoid infiltrate in papillary dermis (40x, H&E), (c) homogenistation of collagen in papillary dermis (40x, H&E)

Click here to view


Therefore diagnosis of generalised non segmental vitiligo with lichen sclerosus was made and patient was treated with topical steroids.


  Discussion Top


The association of LS and acral vitiligo is well documented in the literature, however, the association of LS with generalized vitiligo is not well reported.[3] Attili et al.[4] reviewed 266 vitiligo cases and did not find association with LS. They suggest that association of vitiligo and LS may have been documented due to the clinical misdiagnosis of vitiligoid LS lesions as vitiligo. Association of LS with GV has not been reported in large epidemiological studies. There have been few reports of generalised vitiligo in association with stereotypical LS.[5],[6] In all such reports, the diagnosis of vitiligo was based on clinical impression only and macular depigmentation can be a presentation of LS, i.e. vitiligoid LS. In contrast the diagnosis of our case was both dermoscopically and histopathologically proven. There was development of LS lesions over generalised vitiligo lesions but not over genitalia lesions which was in contrary to cases reported in literature. The dermoscopic and histopathological differences of LS and vitiligo described in [Table 1].[3],[7]
Table 1 Dermoscopic and histopathological differences of LS and vitiligo

Click here to view


Reports of the synchronous onset of LS and vitiligo have supported the relationship between LS and vitiligo. It can be explained by a common pathogenetic mechanism shared by vitiligo and LS as interface dermatitis resulting in depigmentation.[8] It is observed that long standing lesions of vitiligo may result in mild clinical atrophy or sclerosis.[9] Histologic evidence demonstrates that development of vitiligo involves a preceding lichenoid inflammatory reaction that may trigger an autoimmune reaction to melanocytes, decreasing their number. It is possible that epitope spreading as a result of lichenoid inflammation in vitiligo, may result in LS.[10] This hypothesis can explain the development of LS over vitiligo lesions as seen in our patient. The treatment of choice for LS is based on high-potency corticosteroids such as c1obetasol propionate, which however appears to be less effective towards extragenital lesions. Successful treatment with topical retinoids has also been reported, especially for LS unresponsive to ultrapotent topical steroid.[11]

The present case is unusual as LS lesions developed over generalised vitiligo patches.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Neill SM, Lewis FM, Tatnall FM, Cox NH, British Association of Dermatologists. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol 2010;163:672-82.  Back to cited text no. 1
    
2.
Borda JM, Abulafia J, Jaimowich L. Syndrome of circumscribed sclero-atrophies. Derm Ibero Lat Am 1968;3:179-202.  Back to cited text no. 2
    
3.
Attili VR, Attili SK. Acral Vitiligo and Lichen sclerosus − association or a distinct pattern?: a clinical and histopathological review of 15 cases. Indian J Dermatol 2015;60:519.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Attili VR, Attili SK. Vitiligoid lichen sclerosus: a reappraisal. Indian J Dermatol Venereol Leprol 2008;74:118-21.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Attili VR, Attili SK. Lichen sclerosus of lips: a clinical and histopathologic study of 27 cases. Int J Dermatol 2010;49:520-5.  Back to cited text no. 5
    
6.
Borda JM, Balsa RE. Lichen sclerosus et atrophicus of a vitiligoid type. Arch Argent Dermatol 1961;11:419-24.  Back to cited text no. 6
    
7.
Al-Refu K. Dermoscopy is a new diagnostic tool in diagnosis of common hypopigmented macular disease: a descriptive study. Dermatol Reports 2018;11:7916.  Back to cited text no. 7
    
8.
Attili VR, Attili SK. Segmental and generalized vitiligo: both forms demonstrate inflammatory histopathological features and clinical mosaicism. Indian J Dermatol 2013;58:433-8.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Attili VR, Attili SK. Lichenoid inflammation in vitiligo − a clinical and histopathologic review of 210 cases. Int J Dermatol 2008;47:663-9.  Back to cited text no. 9
    
10.
Weisberg EL, Le LQ, Cohen JB. A case of simultaneously occurring lichen sclerosus and segmental vitiligo: connecting the underlying autoimmune pathogenesis. Int J Dermatol 2008;47:1053-5.  Back to cited text no. 10
    
11.
Guerriero C, Manco S, Paradisi A, Capizzi R, Fossati B, Fabrizi G. Extragenital lichen sclerosus and atrophicus treated with topical steroids and retinoids in a child with vitiligo. Int J Immunopathol Pharmacol 2008;21:757-9.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures
Article Tables

 Article Access Statistics
    Viewed74    
    Printed0    
    Emailed0    
    PDF Downloaded6    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]