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CORRESPONDENCE
Year : 2018  |  Volume : 63  |  Issue : 5  |  Page : 427-428
A case of bullous pemphigoid associated with lichen sclerosus et atrophicus


Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan

Date of Web Publication31-Aug-2018

Correspondence Address:
Asuka Yoshifuku
Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_127_18

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How to cite this article:
Yoshifuku A, Okubo A, Jimura N, Fujii K, Higashi Y, Kanekura T. A case of bullous pemphigoid associated with lichen sclerosus et atrophicus. Indian J Dermatol 2018;63:427-8

How to cite this URL:
Yoshifuku A, Okubo A, Jimura N, Fujii K, Higashi Y, Kanekura T. A case of bullous pemphigoid associated with lichen sclerosus et atrophicus. Indian J Dermatol [serial online] 2018 [cited 2020 Jun 2];63:427-8. Available from: http://www.e-ijd.org/text.asp?2018/63/5/427/240092




Sir,

We encountered a 66-year-old woman with bullous pemphigoid (BP) that developed on lichen sclerosus et atrophicus (LSA) lesions.

The patient suffered from LSA for 12 years. While LSA most commonly affects the vulval area, her LSA involved the trunk, extremities, and the anogenital skin.[1] Topical treatment with corticosteroids and tacrolimus yielded limited success.

Tense bullae appeared on the sclerotic LSA lesions in the genital region 12 years after the diagnosis of LSA [Figure 1]a and [Figure 1]b. The bullous lesions spread to the skin on the abdomen and chest affected by LSA. Her serum BP antibody, BP180, was 46.9 U/ml (chemiluminescence enzyme immunoassay). Skin biopsies from her abdomen and genitalia revealed subepidermal cleft with predominant eosinophilic infiltration [Figure 1]c and [Figure 1]d. In the upper dermis, we noted pronounced edema and collagen homogenization [Figure 1]c and [Figure 1]e. Direct immunofluorescence study of the same specimens revealed linear deposits of immunoglobulin G in the basement membrane zone [Figure 1]f. The above findings led to the diagnosis of BP arising on LSA. Oral prednisolone (PSL, 30 mg/day) was started. The bullous lesions improved quickly, and her autoantibodies became negative. The PSL dose was gradually tapered to 10 mg, and she suffered no relapse in 1 year.
Figure 1: Tense bullae and erosions on white glossy sclerotic LSA lesions on the abdomen (a) and genitalia (b). (c) Skin biopsy revealed a subepidermal cleft and infiltration of numerous eosinophils (H and E, ×10). (d) Close-up view of the subepidermal cleft in Figure 1c (H and E, ×200). (e) Close-up view of the upper dermis in Figure 1c (H and E, ×200). Note pronounced edema and collagen homogenization in the upper dermis. (f) Direct immunofluorescence showed linear deposits of immunoglobulin G in the basement membrane zone (×200)

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Although the etiology and pathogenesis of LSA are not fully understood, they are thought to be multifactorial with the involvement of autoimmune-related factors.[2],[3],[4] Our review of the English literature found only three cases of BP associated with LSA.[2],[3],[4],[5] Meyrick Thomas et al.[2] who investigated autoimmune-related phenomena in 350 women with histologically confirmed LSA demonstrated that 21.5% had one or more autoimmune diseases including alopecia areata (9%), vitiligo (6%), hypothyroidism (6%), and BP (1%); 42% presented with an autoantibody. With respect to autoantibodies against BP antigens, Baldo et al.[3] reported that the noncollagenous 16A domain of BP180 was a target for circulating T-cells in over 40% of their patients with vulval lichen sclerosus or lichen planus; 4 of 19 manifested associated autoantibodies to BP180. A lichen sclerosus cohort study of 149 patients revealed elevated anti-BP antibodies in 5 (3.4%), BP180 in 4, and BP230 in one patient.[4] These studies suggested an immunogenetic connection between LSA and anti-BP autoantibodies. Ours was a rare case of BP with LSA and further studies to examine the pathological role of anti-BP autoantibodies produced in LSA patients who develop associated BP are needed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kawamura E, Kanekura T, Mera Y, Uchimiya H, Kanzaki T. Generalized lichen sclerosus et atrophicus: Report of a case. J Dermatol 2005;32:1048-50.  Back to cited text no. 1
    
2.
Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. Lichen sclerosus et atrophicus and autoimmunity – A study of 350 women. Br J Dermatol 1988;118:41-6.  Back to cited text no. 2
    
3.
Baldo M, Bailey A, Bhogal B, Groves RW, Ogg G, Wojnarowska F, et al. T cells reactive with the NC16A domain of BP180 are present in vulval lichen sclerosus and lichen planus. J Eur Acad Dermatol Venereol 2010;24:186-90.  Back to cited text no. 3
    
4.
Gambichler T, Höxtermann S, Skrygan M, Eberz B, Regauer S, Scola N, et al. Occurrence of circulating anti-bullous pemphigoid antibodies in patients with lichen sclerosus. J Eur Acad Dermatol Venereol 2011;25:369-70.  Back to cited text no. 4
    
5.
Walsh ML, Leonard N, Shawki H, Bell HK. Lichen sclerosus and immunobullous disease. J Low Genit Tract Dis 2012;16:468-70.  Back to cited text no. 5
    


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