|Year : 2021 | Volume
| Issue : 1 | Page : 55-58
The convergence of extragenital lichen sclerosus with generalized vitiligo – an unusual presentation
Khushboo Arora1, Aditi Dhanta1, Neirita Hazarika1, Prashant Joshi2
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 Submission||11-Feb-2020|
|Date of Decision||11-Apr-2020|
|Date of Acceptance||27-Jul-2020|
|Date of Web Publication||07-Apr-2021|
MD Aditi Dhanta
Department of Dermatology, Venereology and Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
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. 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. There have been a few reported cases in the literature of the coexistence of acrofacial vitiligo and LS.
| Case Report|| |
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|
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|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|
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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)|
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Therefore diagnosis of generalised non segmental vitiligo with lichen sclerosus was made and patient was treated with topical steroids.
| Discussion|| |
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. Attili et al. 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., 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].,
|Table 1 Dermoscopic and histopathological differences of LS and vitiligo|
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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. It is observed that long standing lesions of vitiligo may result in mild clinical atrophy or sclerosis. 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. 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.
The present case is unusual as LS lesions developed over generalised vitiligo patches.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]