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Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus
DERMATOLOGICA SINICA xxx (2013) 1e3
Contents lists available at SciVerse ScienceDirect
Dermatologica Sinica journal homepage: http://www.derm-sinica.com
CASE REPORT
Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus Han Su Kim, Heung Yeol Kim, Eun Joo Park*, Kwang Ho Kim, Kwang Joong Kim Department of Dermatology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang-si, Gyeonggi-do, South Korea
a r t i c l e i n f o
a b s t r a c t
Article history: Received: Dec 20, 2012 Revised: May 16, 2013 Accepted: May 17, 2013
Vulvitis circumscripta plasmacellularis (VCP) is a rare, benign vulvar disorder that presents with erythematous patches and erosions. Patients with VCP usually experience vulvodynia, pruritus, or dyspareunia, which may cause severe discomfort. A 72-year-old woman presented with 10 years of vulvar pruritus and whitish patches on the labia minora and majora. A biopsy was performed on the labia minora and lichen sclerosus was diagnosed. Her condition improved after treatment with a topical steroid. After 5 months, however, her symptoms reappeared with aggravated skin lesions. A rebiopsy was performed and VCP was diagnosed. There have only been two reported cases of concurrence of VCP with lichen sclerosus. We herein report an unusual case of VCP in pre-existing lichen sclerosus. Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: lichen sclerosus vulvitis circumscripta plasmacellularis
Introduction Vulvitis circumscripta plasmacellularis (VCP) was first described by Garnier1 in 1954. It is a relatively rare chronic inflammatory disease. It is clinically and histopathologically similar to balanitis circumscripta plasmacellularis in males. Clinical symptoms of VCP are vulvodynia, pruritus, or dyspareunia, but VCP may be asymptomatic.2 Only two cases have been reported on concurrent VCP and vulvar lichen sclerosus.2,3 In this report, we describe an unusual case of VCP in pre-existing lichen sclerosus. Case report A 72-year-old woman presented with 10 years of vulvar pruritus. She had localized whitish patches on the labia minora and majora (Figure 1A). Her medical history included hypertension for 1 year. Laboratory test results were within normal limits. A biopsy was performed on the labia minora, including the majora, the results of which revealed pronounced edema and homogenization of collagen in the upper dermis. Hyperkeratosis and follicular plugging in the epidermis and inflammatory infiltrate in the middermis were observed (Figure 2). Lichen sclerosus was diagnosed, and the patient was treated with a topical potent corticosteroid (clobetasol-17-propionate) for 2 weeks. Her condition improved,
* Corresponding author. Department of Dermatology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, 896 Pyeongchon-dong, Dongan-gu, Anyang-si, Gyeonggi-do 431-070, South Korea. Tel.: þ82 31 380 3765; fax: þ82 31 386 3761. E-mail addresses: [email protected], [email protected] (E.J. Park).
but her symptoms were aggravated after 5 months. She presented with severe pruritus and pain in the vulvar area. A physical examination revealed localized whitish to erythematous eroded patches on the labia minora (Figure 1B). A rebiopsy on the eroded patches of the labia minora was performed to rule out squamous cell carcinoma. The biopsy specimen showed epidermal thinning, absence of the stratum corneum, irregular acanthosis, and spongiosis in the epidermis. A dense dermal lichenoid infiltrate with many plasma cells and lymphocytes was observed (Figure 3). Results of an immunohistochemical analysis revealed that plasma cells were polyclonal for kappa and lambda light chains, and syphilis testing with venereal disease research laboratory was negative. Based on the results of the biopsy, VCP was diagnosed. After treatment with a systemic and topical corticosteroid, the patient’s condition temporarily improved; however, her symptoms recurred, and she was advised to apply imiquimod 5% cream two to three times a week. After 1 month, her condition slightly improved and she was lost to follow-up. Discussion VCP is a rare vulvar disorder, which causes vulvodynia, pruritus, and discomfort. Patients usually present with erythematous patches or plaques on the labia minora, majora, and other vulvar structures. The clinical appearance of VCP may be similar to extramammary Paget’s disease, squamous cell carcinoma, Bowen’s disease, pemphigus vulgaris, allergic contact dermatitis, candidiasis, syphilis, sexual abuse, herpes simplex infection, erosive lichen planus, or fixed drug eruption.2,4 The etiology and pathogenesis of VCP remain unclear, but predisposing factors, such as infection,
1027-8117/$ e see front matter Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.dsi.2013.05.003
Please cite this article in press as: Kim HS, et al., Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus, Dermatologica Sinica (2013), http://dx.doi.org/10.1016/j.dsi.2013.05.003
2
H.S. Kim et al. / Dermatologica Sinica xxx (2013) 1e3
Figure 1 (A) Localized whitish patches on the labia minora and majora. (B) After 5 months, the patient’s symptoms were aggravated. She presented with localized whitish to erythematous eroded patches on the labia minora; a rebiopsy was performed on the eroded patches of labia minora (arrow).
Figure 2 Biopsy specimen from the labia minora including the majora shows pronounced edema and homogenization of collagen in the upper dermis. Hyperkeratosis and follicular plugging in the epidermis and inflammatory infiltrate in the mid-dermis are observed (hematoxylin and eosin, 40).
poor hygiene, heat, and constant friction have been suggested.5,6 In our patient’s case, due to the pruritic characteristic (which may trigger friction) of lichen sclerosus, the cause of VCP may have been lichen sclerosus. Histological findings of VCP are as follows.2,7,8 The epidermis shows atrophy and often an absence of the horny and granular cell layers. It is composed of lozenge-shaped keratinocytes, and spongiosis in the epidermis is also present. The dermis shows a lichenoid infiltrate predominantly composed of plasma cells. In addition, blood vessels are dilated, and there may be extravasation of erythrocytes and deposits of hemosiderin. Reactive epithelial changes may be present, but true dysplasia is absent. In our patient’s case, VCP was diagnosed in pre-existing lichen sclerosus. The concurrence of VCP with vulvar lichen sclerosus has been reported only twice, one of which involved a 60-year-old woman, who presented with a 1-year history of vulvodynia. Results of a biopsy confirmed the diagnosis of lichen sclerosus and VCP.2 The other case was a 60-year-old woman with a long-standing history of lichen sclerosus under local therapy; she developed soreness and a scaling erythematous lesion in the vaginal introitus. A biopsy confirmed the diagnosis of VCP.3 As a complication of long-standing lichen sclerosus, the risk of developing squamous cell carcinoma is in the range of 4e5%, and lichen sclerosus is also associated with genital verrucous carcinoma.9,10 Therefore, a biopsy should be performed when lichen sclerosus lasts for a long period and shows erythematous eroded lesion. However, we note that similar lesions may be exhibited by other diseases, such as VCP. Herein, we described an interesting and rare case of VCP in pre-existing lichen sclerosus. Dermatologists
Figure 3 Rebiopsy specimen from the eroded patch of the labia minora shows epidermal thinning, absence of the stratum corneum, irregular acanthosis, and spongiosis in the epidermis. A dense dermal lichenoid infiltrate with many plasma cells and lymphocytes is observed [hematoxylin and eosin: (A) 100; (B) 400].
Please cite this article in press as: Kim HS, et al., Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus, Dermatologica Sinica (2013), http://dx.doi.org/10.1016/j.dsi.2013.05.003
H.S. Kim et al. / Dermatologica Sinica xxx (2013) 1e3
should understand that VCP can result from lichen sclerosus and that it resembles squamous cell carcinoma. References 1. Garnier G. Vulvite erythemateuse circonscrite benign a type erytheroplasique. Bull Soc Fr Dermatol Syphiligr 1954;61:102e4. 2. van Kessel MA, van Lingen RG, Bovenschen HJ. Vulvitis plasmacellularis circumscripta in pre-existing lichen sclerosus: treatment with imiquimod 5% cream. J Am Acad Dermatol 2010;63:11e3. 3. Fernández-Aceñero MJ, Córdova S. Zoon’s vulvitis (vulvitis circumscripta plasmacellularis). Arch Gynecol Obstet 2010;282:351e2. 4. Albers SE, Taylor G, Huyer D, Oliver G, Krafchik BR. Vulvitis circumscripta plasmacellularis mimicking child abuse. J Am Acad Dermatol 2000;42:1078e80.
3
5. Patton LW, Elgart ML, Williams CM. Vulvar erythema and induration. Extraintestinal Crohn’s disease of the vulva. Arch Dermatol 1990;126:1351e2. 1354e5. 6. Kuniyuki S, Asada T, Yasumoto R. A case of vulvitis circumscripta plasmacellularis positive for herpes simplex type II antigen. Clin Exp Dermatol 1998;23:230e1. 7. Solt I, Lowenstein L, Amit A, Bergman R, Kerner H. Ulcerative vulvitis circumscripta plasmacellularis. Isr Med Assoc J 2004;6:117e8. 8. Botros SM, Dieterich M, Sand PK, Goldberg RP. Successful treatment of Zoon’s vulvitis with high potency topical steroid. Int Urogynecol J Pelvic Floor Dysfunct 2006;17:178e9. 9. Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999;353:1777e83. 10. Wang SH, Chi CC, Wong YW, Salim A, Manek S, Wojnarowska F. Genital verrucous carcinoma is associated with lichen sclerosus: a retrospective study and review of the literature. J Eur Acad Dermatol Venereol 2010;24: 815e9.
Please cite this article in press as: Kim HS, et al., Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus, Dermatologica Sinica (2013), http://dx.doi.org/10.1016/j.dsi.2013.05.003
Contents lists available at SciVerse ScienceDirect
Dermatologica Sinica journal homepage: http://www.derm-sinica.com
CASE REPORT
Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus Han Su Kim, Heung Yeol Kim, Eun Joo Park*, Kwang Ho Kim, Kwang Joong Kim Department of Dermatology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang-si, Gyeonggi-do, South Korea
a r t i c l e i n f o
a b s t r a c t
Article history: Received: Dec 20, 2012 Revised: May 16, 2013 Accepted: May 17, 2013
Vulvitis circumscripta plasmacellularis (VCP) is a rare, benign vulvar disorder that presents with erythematous patches and erosions. Patients with VCP usually experience vulvodynia, pruritus, or dyspareunia, which may cause severe discomfort. A 72-year-old woman presented with 10 years of vulvar pruritus and whitish patches on the labia minora and majora. A biopsy was performed on the labia minora and lichen sclerosus was diagnosed. Her condition improved after treatment with a topical steroid. After 5 months, however, her symptoms reappeared with aggravated skin lesions. A rebiopsy was performed and VCP was diagnosed. There have only been two reported cases of concurrence of VCP with lichen sclerosus. We herein report an unusual case of VCP in pre-existing lichen sclerosus. Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: lichen sclerosus vulvitis circumscripta plasmacellularis
Introduction Vulvitis circumscripta plasmacellularis (VCP) was first described by Garnier1 in 1954. It is a relatively rare chronic inflammatory disease. It is clinically and histopathologically similar to balanitis circumscripta plasmacellularis in males. Clinical symptoms of VCP are vulvodynia, pruritus, or dyspareunia, but VCP may be asymptomatic.2 Only two cases have been reported on concurrent VCP and vulvar lichen sclerosus.2,3 In this report, we describe an unusual case of VCP in pre-existing lichen sclerosus. Case report A 72-year-old woman presented with 10 years of vulvar pruritus. She had localized whitish patches on the labia minora and majora (Figure 1A). Her medical history included hypertension for 1 year. Laboratory test results were within normal limits. A biopsy was performed on the labia minora, including the majora, the results of which revealed pronounced edema and homogenization of collagen in the upper dermis. Hyperkeratosis and follicular plugging in the epidermis and inflammatory infiltrate in the middermis were observed (Figure 2). Lichen sclerosus was diagnosed, and the patient was treated with a topical potent corticosteroid (clobetasol-17-propionate) for 2 weeks. Her condition improved,
* Corresponding author. Department of Dermatology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, 896 Pyeongchon-dong, Dongan-gu, Anyang-si, Gyeonggi-do 431-070, South Korea. Tel.: þ82 31 380 3765; fax: þ82 31 386 3761. E-mail addresses: [email protected], [email protected] (E.J. Park).
but her symptoms were aggravated after 5 months. She presented with severe pruritus and pain in the vulvar area. A physical examination revealed localized whitish to erythematous eroded patches on the labia minora (Figure 1B). A rebiopsy on the eroded patches of the labia minora was performed to rule out squamous cell carcinoma. The biopsy specimen showed epidermal thinning, absence of the stratum corneum, irregular acanthosis, and spongiosis in the epidermis. A dense dermal lichenoid infiltrate with many plasma cells and lymphocytes was observed (Figure 3). Results of an immunohistochemical analysis revealed that plasma cells were polyclonal for kappa and lambda light chains, and syphilis testing with venereal disease research laboratory was negative. Based on the results of the biopsy, VCP was diagnosed. After treatment with a systemic and topical corticosteroid, the patient’s condition temporarily improved; however, her symptoms recurred, and she was advised to apply imiquimod 5% cream two to three times a week. After 1 month, her condition slightly improved and she was lost to follow-up. Discussion VCP is a rare vulvar disorder, which causes vulvodynia, pruritus, and discomfort. Patients usually present with erythematous patches or plaques on the labia minora, majora, and other vulvar structures. The clinical appearance of VCP may be similar to extramammary Paget’s disease, squamous cell carcinoma, Bowen’s disease, pemphigus vulgaris, allergic contact dermatitis, candidiasis, syphilis, sexual abuse, herpes simplex infection, erosive lichen planus, or fixed drug eruption.2,4 The etiology and pathogenesis of VCP remain unclear, but predisposing factors, such as infection,
1027-8117/$ e see front matter Copyright Ó 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.dsi.2013.05.003
Please cite this article in press as: Kim HS, et al., Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus, Dermatologica Sinica (2013), http://dx.doi.org/10.1016/j.dsi.2013.05.003
2
H.S. Kim et al. / Dermatologica Sinica xxx (2013) 1e3
Figure 1 (A) Localized whitish patches on the labia minora and majora. (B) After 5 months, the patient’s symptoms were aggravated. She presented with localized whitish to erythematous eroded patches on the labia minora; a rebiopsy was performed on the eroded patches of labia minora (arrow).
Figure 2 Biopsy specimen from the labia minora including the majora shows pronounced edema and homogenization of collagen in the upper dermis. Hyperkeratosis and follicular plugging in the epidermis and inflammatory infiltrate in the mid-dermis are observed (hematoxylin and eosin, 40).
poor hygiene, heat, and constant friction have been suggested.5,6 In our patient’s case, due to the pruritic characteristic (which may trigger friction) of lichen sclerosus, the cause of VCP may have been lichen sclerosus. Histological findings of VCP are as follows.2,7,8 The epidermis shows atrophy and often an absence of the horny and granular cell layers. It is composed of lozenge-shaped keratinocytes, and spongiosis in the epidermis is also present. The dermis shows a lichenoid infiltrate predominantly composed of plasma cells. In addition, blood vessels are dilated, and there may be extravasation of erythrocytes and deposits of hemosiderin. Reactive epithelial changes may be present, but true dysplasia is absent. In our patient’s case, VCP was diagnosed in pre-existing lichen sclerosus. The concurrence of VCP with vulvar lichen sclerosus has been reported only twice, one of which involved a 60-year-old woman, who presented with a 1-year history of vulvodynia. Results of a biopsy confirmed the diagnosis of lichen sclerosus and VCP.2 The other case was a 60-year-old woman with a long-standing history of lichen sclerosus under local therapy; she developed soreness and a scaling erythematous lesion in the vaginal introitus. A biopsy confirmed the diagnosis of VCP.3 As a complication of long-standing lichen sclerosus, the risk of developing squamous cell carcinoma is in the range of 4e5%, and lichen sclerosus is also associated with genital verrucous carcinoma.9,10 Therefore, a biopsy should be performed when lichen sclerosus lasts for a long period and shows erythematous eroded lesion. However, we note that similar lesions may be exhibited by other diseases, such as VCP. Herein, we described an interesting and rare case of VCP in pre-existing lichen sclerosus. Dermatologists
Figure 3 Rebiopsy specimen from the eroded patch of the labia minora shows epidermal thinning, absence of the stratum corneum, irregular acanthosis, and spongiosis in the epidermis. A dense dermal lichenoid infiltrate with many plasma cells and lymphocytes is observed [hematoxylin and eosin: (A) 100; (B) 400].
Please cite this article in press as: Kim HS, et al., Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus, Dermatologica Sinica (2013), http://dx.doi.org/10.1016/j.dsi.2013.05.003
H.S. Kim et al. / Dermatologica Sinica xxx (2013) 1e3
should understand that VCP can result from lichen sclerosus and that it resembles squamous cell carcinoma. References 1. Garnier G. Vulvite erythemateuse circonscrite benign a type erytheroplasique. Bull Soc Fr Dermatol Syphiligr 1954;61:102e4. 2. van Kessel MA, van Lingen RG, Bovenschen HJ. Vulvitis plasmacellularis circumscripta in pre-existing lichen sclerosus: treatment with imiquimod 5% cream. J Am Acad Dermatol 2010;63:11e3. 3. Fernández-Aceñero MJ, Córdova S. Zoon’s vulvitis (vulvitis circumscripta plasmacellularis). Arch Gynecol Obstet 2010;282:351e2. 4. Albers SE, Taylor G, Huyer D, Oliver G, Krafchik BR. Vulvitis circumscripta plasmacellularis mimicking child abuse. J Am Acad Dermatol 2000;42:1078e80.
3
5. Patton LW, Elgart ML, Williams CM. Vulvar erythema and induration. Extraintestinal Crohn’s disease of the vulva. Arch Dermatol 1990;126:1351e2. 1354e5. 6. Kuniyuki S, Asada T, Yasumoto R. A case of vulvitis circumscripta plasmacellularis positive for herpes simplex type II antigen. Clin Exp Dermatol 1998;23:230e1. 7. Solt I, Lowenstein L, Amit A, Bergman R, Kerner H. Ulcerative vulvitis circumscripta plasmacellularis. Isr Med Assoc J 2004;6:117e8. 8. Botros SM, Dieterich M, Sand PK, Goldberg RP. Successful treatment of Zoon’s vulvitis with high potency topical steroid. Int Urogynecol J Pelvic Floor Dysfunct 2006;17:178e9. 9. Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999;353:1777e83. 10. Wang SH, Chi CC, Wong YW, Salim A, Manek S, Wojnarowska F. Genital verrucous carcinoma is associated with lichen sclerosus: a retrospective study and review of the literature. J Eur Acad Dermatol Venereol 2010;24: 815e9.
Please cite this article in press as: Kim HS, et al., Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus, Dermatologica Sinica (2013), http://dx.doi.org/10.1016/j.dsi.2013.05.003