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Australasian Journal of Dermatology (2015) ••, ••–••
doi: 10.1111/ajd.12355
ORIGINAL RESEARCH
Diagnostic criteria in 72 women with erosive vulvovaginal lichen planus Harriet Cheng,1 Amanda Oakley,1 Darion Rowan2 and Duncan Lamont3 1
Department of Dermatology, Waikato Hospital, 3Department of Pathology, Waikato Hospital, Hamilton, and 2 Department of Gynaecology Oncology, National Womens Health, Auckland, New Zealand
ABSTRACT Background: Erosive vulvovaginal lichen planus (EVLP) is a chronic, painful dermatosis affecting mucocutaneous sites. Clinicopathological diagnostic criteria have been described on the basis of expert consensus. The aim of this study was to review the presentation of EVLP, particularly assessing the frequency of suggested diagnostic criteria. Methods: Clinical signs, symptoms and histological features of women with a clinical diagnosis of EVLP were identified from clinical records and photographs. Results: In all, 72 women with an average age of 67 years were included. Pain or burning were documented in 66/72 cases (92%) and itch in 36 (50%). Clinical images showed well-demarcated red shiny areas or erosions at the vaginal introitus (96%), scarring with loss of normal architecture (88%) and hyperkeratotic border and Wickham striae (46%). A total of 27 women had mucosal disease at another site (38%) and 24 had vaginal involvement (33%). Vulval histology was available for 45/72 cases (63%). The most prevalent histological finding was a band of inflammation with predominant lymphocytes (35/72, 49%). Overall, 97% of cases had at least three of nine suggested diagnostic criteria. Conclusions: The most frequent findings in women with EVLP were symptoms of pain or burning, well-demarcated red shiny areas or erosions at the introitus and scarring with loss of architecture. Our findings support the recently described diagnostic criteria for EVLP.
Correspondence: Dr Amanda Oakley, Dermatologist, Waikato Hospital, Private bag 3200, Hamilton 3240, New Zealand. Email: [email protected] Harriet Cheng, MBChB. Amanda Oakley, FRACP. Darion Rowan, FACD. Duncan Lamont, M Med, Path (UCT). Conflict of interest: none. Submitted 25 January 2015; accepted 24 April 2015. © 2015 The Australasian College of Dermatologists
Key words: diagnostic criteria, erosive vulvovaginal lichen planus, genital lichen planus.
INTRODUCTION Lichen planus is a chronic inflammatory disorder affecting skin and mucous membranes including the vagina, vulva, oral cavity, ear canals, oesophagus and lacrimal ducts. Its overall prevalence is unknown but is estimated to be around 1%.1 The vulvovaginal form is rare and may occur in isolation or in combination with oral disease (the vulvovaginal gingival syndrome2) or other mucocutaneous disease. Vulvovaginal disease usually results in erosions leading to chronic vulval pain, difficulty with or complete cessation of sexual activity and discomfort on micturition and defecation. Other reported symptoms include itch, vaginal discharge and bleeding.3 The long-term risk of associated vulval carcinoma remains unclear.4 Adding to the burden of disease, response to treatment is often disappointing and the clinical course chronic, irrespective of therapy.5 A recent review of mucosal lichen planus by the Cochrane Collaboration found only weak evidence for the effectiveness of any of the treatments for oral disease and did not find any studies of genital disease.6 There have been several retrospective studies documenting clinical symptoms and signs in erosive vulvovaginal lichen planus (EVLP).2,3,7 Commonly reported clinical findings include sharply demarcated erythematous lesions or erosions, hyperkeratotic areas, Wickham striae (fine white lines over the surface of lesions) and labial fusion leading to introital stenosis (Fig. 1). A single prospective cohort study found vulval pain was reported in 80% and vulval erosions were present in 97%.8
Abbreviation: EVLP
erosive vulvovaginal lichen planus
2
H Cheng et al. Table 2 Frequency of symptoms and signs in women with erosive vulvovaginal lichen planus
Symptoms Pain or burning Itch Clinical signs Well-demarcated erosions or erythematous areas at the vaginal introitus Scarring or loss of architecture White plaques Hyperkeratotic border to lesions or Wickham striae Involvement of other mucosal sites Vaginal inflammation Papules
Figure 1 Characteristic clinical findings in erosive vulvovaginal lichen planus. Well-defined introital erosion and extensive architectural change with labial resorption, buried clitoris and introital stenosis. Table 1
Diagnostic criteria for erosive vulvovaginal lichen planus9
Well-demarcated erosions or erythematous areas at the vaginal introitus Presence of a hyperkeratotic border to lesions or Wickham striae in surrounding skin Symptoms of pain or burning Scarring or loss of normal architecture Presence of vaginal inflammation Involvement of other mucosal surfaces Presence of a well-defined inflammatory band involving the dermoepidermal junction Presence of an inflammatory band consisting predominantly of lymphocytes Signs of basal layer degeneration
Recently, clinicopathological diagnostic criteria have been suggested following an electronic Delphi consensus exercise involving experts in the diagnosis and management of vulval disease.9 Nine supportive diagnostic criteria reached the consensus (Table 1). It was suggested that at least three supportive features should be present to diagnose EVLP. The aim of this study was to review the clinical and histological features of EVLP in a cohort of women from New Zealand in particular, to assess the frequency of suggested diagnostic criteria.
MATERIALS AND METHODS New Zealand Health and Disability Ethics Committee review was not required, as this was a retrospective obser© 2015 The Australasian College of Dermatologists
Frequency (n = 72)
%
66 36
92 50
69
96
63 32 33
88 50 46
27 24 2
38 33 3
vational study with minimal risk. Women with a clinical diagnosis of EVLP who had attended specialist dermatology clinics at two sites in New Zealand from September 2002 to November 2013 were included. Cases where EVLP was not the primary skin disorder or where there was uncertainty of the diagnosis were excluded. Cases where clinical images were of poor quality were excluded. Written consent had been obtained for clinical photographs to be used in a research setting. Clinical signs were identified from photographs by a dermatologist with expertise in vulval skin disease. Symptoms and clinical signs that were not visible in photographs (e.g. vaginal involvement, other mucosal disease) were identified from clinical notes. Histological reports were reviewed where available.
RESULTS In total, 89 women were identified and of these 17 were excluded; nine due to uncertainty of EVLP as the primary diagnosis, five due to the poor quality of the clinical images and three due to insufficient clinical information. A total of 72 women were included in the final analysis with an average age of 67 years (range 26–93). Clinical photographs were available for all patients. Frequency of symptoms and signs are shown in Table 2. Accompanying disease at other mucosal sites was found in 36% of the participants. Most frequently this was oral (23/ 72) or anal (nine). Two women had oesophageal disease. Architectural changes included labial resorption (56), buried clitoris (21) and introital stenosis (18). A vulval biopsy was performed in 55/72 women (76%) and 45 histology reports were accessed. Histological findings included a band of inflammation with predominant lymphocytes (35/45), a well-defined inflammatory band at the dermoepidermal junction (32) and evidence of basal layer degeneration (27) (Fig. 2). Six cases (13%) displayed non-specific findings with none of the histological features suggested as diagnostic criteria by Simpson and colleagues. No other inflammatory disorder or neoplasia was reported in these biopsies.
Erosive vulvovaginal lichen planus Of the diagnostic criteria suggested by Simpson and colleagues, the most common were well-demarcated erosions or erythematous areas at the vaginal introitus (96%), symptoms of pain or burning (92%) and scarring or loss of architecture (88%). The median number of diagnostic criteria met was five. Overall, 70 patients met at least three of the nine diagnostic criteria (97%); 59 patients met at least four diagnostic criteria (82%) (Fig. 3).
DISCUSSION EVLP is a painful and chronic genital dermatosis. Women often present late, after months or years of symptoms or misdiagnosis. In our group, pain was by far the most commonly documented symptom. Of the cases where pain or burning was not documented, clinical notes often stated
Figure 2 Histological features of erosive vulvovaginal lichen planus. Inflammatory infiltrate with lymphocyte predominance at the dermoepidermal junction. Degeneration of the epidermal basal layer, arrow shows necrotic keratinocyte (H–E ×200).
3
discomfort or other vague descriptors. A number of studies have documented pain or soreness as a common presenting symptom in EVLP.3,7,8 A retrospective study of 95 women with EVLP in The Netherlands found the most common presenting symptoms to be dyspareunia (54%), vulval soreness or burning (50%) and pruritus (48%).3 Similar to our findings, sharply demarcated erythematous lesions were the most common clinical finding (81%). Overall, 114 women were included in a prospective cohort study in Oxford, England that examined clinical symptoms and signs of vulval lichen planus and response to treatment.8 Again, vulval pain and pruritus were the most common symptoms (80 and 65%, respectively). Vulval erosions were found in 97% of cases. Just over half of cases had vaginal involvement (58%). Clitoral burying and introital narrowing were common (68 and 59%, respectively). These findings, combined with our own suggest that welldemarcated red areas or erosions are the most common clinical finding in EVLP. However, these may not be specific. Erosions are areas with partial epidermal loss and are also found in lichen sclerosus, which is a much more common genital dermatosis. Symmetrical erosions at the introitus are suggestive of EVLP. Introital stenosis and loss of architecture are also features shared with lichen sclerosus. Lichen planus and lichen sclerosus have overlapping histological features, particularly in the early stages. A band of inflammation at the dermoepidermal junction can be found in both disorders. The additional finding of homogenisation of collagen in the superficial dermis is suggestive of lichen sclerosus. When it is difficult to differentiate between the two conditions, both clinically and histologically, a dual diagnosis can be considered. Over time, women with lichen sclerosus develop shiny, hypopigmented and sclerotic skin. Vaginal involvement is rare in lichen sclerosus but has been reported in association with severe disease and uterine prolapse.10 Vulval disease due to oestrogen deficiency, mucous membrane pemphigoid, fixed drug eruption, plasma cell vulvitis
Figure 3 Number of diagnostic criteria present in 72 women with erosive vulvovaginal lichen planus. © 2015 The Australasian College of Dermatologists
4
H Cheng et al.
and graft versus host disease may also be difficult to distinguish from EVLP on clinical grounds alone and diagnosis requires vigilance. These conditions often have distinctive findings on histological examination and immunofluorescence. The nine recently published diagnostic criteria for EVLP were the outcome of an electronic Delphi consensus exercise.9 It was suggested that at least three criteria should be met to be confident in a diagnosis. However, this number is up for debate; a number of experts considered four to be a more appropriate number, presumably to reduce false positives (particularly in women with lichen sclerosus). In all, 97% of the women in our study met at least three diagnostic criteria. The remaining two women met two criteria. Of these, one had red shiny areas at the introitus; however, they were poorly demarcated. The biopsy report for the second woman was unavailable for review. A diagnosis of EVLP may be made on clinical grounds alone, although a biopsy should be undertaken where there is diagnostic uncertainty or suspicion of neoplasia. A biopsy was performed in nearly three-quarters of the patients in our group. Of available reports, 84% of the biopsies met at least one of the histological criteria suggested. Histopathological findings in EVLP include epidermal ulceration with the typical features of lichen planus at the edge of the lesions (Fig. 2). The infiltrate often contains lymphocytes, although inflammation may be sparse in endstage disease. Plasma cells are also a frequent finding in genital sites. A lichenoid infiltrate with plasma cells is also found in plasma cell vulvitis. Red cell extravasation, dermal fibrosis and a relative abundance of plasma cells can help to differentiate plasma cell vulvitis from EVLP histologically.11 The main limitation of this study is its retrospective design. Patients who were included had a firm clinical diagnosis of EVLP and therefore we cannot comment on the usefulness of the diagnostic criteria in more difficult cases. Further studies are required to validate diagnostic criteria in real-life clinical setting, particularly in differentiating between EVLP and other vulval dermatoses. Another limitation is the lack of histology for some cases. Ideally, for the benefit of research, histological diagnosis should be available for all patients. However, previous studies have shown that histological findings in EVLP can be variable, including chronic inflammation, lichen sclerosus and other non-
© 2015 The Australasian College of Dermatologists
specific findings.3 Because of this we also included women with a clinical diagnosis of EVLP made by expert clinicians with many years of experience in vulval dermatology. In summary, symptoms of pain or burning, welldemarcated red shiny areas or erosions at the introitus and scarring or loss of architecture were the most common findings and were present in most of the women with erosive lichen planus. Our findings support the diagnostic criteria suggested by Simpson and colleagues. Most patients met at least three diagnostic criteria. A validation of the EVLP diagnostic criteria with prospective studies is required.
REFERENCES 1.
2.
3.
4. 5.
6.
7.
8.
9.
10.
11.
Eisen D. The clinical features, malignant potential, and systemic associations of oral lichen planus: a study of 723 patients. J. Am. Acad. Dermatol. 2002; 46: 207–14. Pelisse M, Leibowitch M, Sedel D et al. A new vulvovaginogingival syndrome. Plurimucous erosive lichen planus. Ann. Dermatol. Venereol. 1982; 109: 797–8. Santegoets L, Helmerhorst T, van der Meijden W. A retrospective study of 95 women with a clinical diagnosis of genital lichen planus. J. Low. Gen. Tract. Dis. 2010; 14: 323–8. Simpson R, Murphy R. Is vulval erosive lichen planus a premalignant condition? Arch. Dermatol. 2012; 148: 1314–6. Cribier B, Fraces C, Chosidow O. Treatment of lichen planus. An evidence based medicine analysis of efficacy. Arch. Dermatol. 1998; 134: 1521–30. Cheng S, Kirtschig G, Cooper S et al. Interventions for erosive lichen planus affecting mucosal sites. Cochrane Database Syst. Rev. 2012: CD008092. Bradford J, Fischer G. Management of vulvovaginal lichen planus: a new approach. J. Low. Genit. Tract Dis. 2012; 17: 28–32. Cooper S, Wojnarowska F. Influence of treatment of erosive lichen planus of the vulva on its prognosis. Arch. Dermatol. 2006; 142: 289–94. Simpson RC, Thomas KS, Leighton P et al. Diagnostic criteria for erosive lichen planus affecting the vulva: an international electronic-Delphi consensus exercise. Br. J. Dermatol. 2013; 169: 337–43. Kendell K, Edwards L. Lichen sclerosus with vaginal involvement: report of 2 cases and review of the literature. JAMA Dermatol. 2013; 149: 1199–202. Weedon D. Skin Pathology, 2nd edn. New York: Churchill Living stone, 2010.
Australasian Journal of Dermatology (2015) ••, ••–••
doi: 10.1111/ajd.12355
ORIGINAL RESEARCH
Diagnostic criteria in 72 women with erosive vulvovaginal lichen planus Harriet Cheng,1 Amanda Oakley,1 Darion Rowan2 and Duncan Lamont3 1
Department of Dermatology, Waikato Hospital, 3Department of Pathology, Waikato Hospital, Hamilton, and 2 Department of Gynaecology Oncology, National Womens Health, Auckland, New Zealand
ABSTRACT Background: Erosive vulvovaginal lichen planus (EVLP) is a chronic, painful dermatosis affecting mucocutaneous sites. Clinicopathological diagnostic criteria have been described on the basis of expert consensus. The aim of this study was to review the presentation of EVLP, particularly assessing the frequency of suggested diagnostic criteria. Methods: Clinical signs, symptoms and histological features of women with a clinical diagnosis of EVLP were identified from clinical records and photographs. Results: In all, 72 women with an average age of 67 years were included. Pain or burning were documented in 66/72 cases (92%) and itch in 36 (50%). Clinical images showed well-demarcated red shiny areas or erosions at the vaginal introitus (96%), scarring with loss of normal architecture (88%) and hyperkeratotic border and Wickham striae (46%). A total of 27 women had mucosal disease at another site (38%) and 24 had vaginal involvement (33%). Vulval histology was available for 45/72 cases (63%). The most prevalent histological finding was a band of inflammation with predominant lymphocytes (35/72, 49%). Overall, 97% of cases had at least three of nine suggested diagnostic criteria. Conclusions: The most frequent findings in women with EVLP were symptoms of pain or burning, well-demarcated red shiny areas or erosions at the introitus and scarring with loss of architecture. Our findings support the recently described diagnostic criteria for EVLP.
Correspondence: Dr Amanda Oakley, Dermatologist, Waikato Hospital, Private bag 3200, Hamilton 3240, New Zealand. Email: [email protected] Harriet Cheng, MBChB. Amanda Oakley, FRACP. Darion Rowan, FACD. Duncan Lamont, M Med, Path (UCT). Conflict of interest: none. Submitted 25 January 2015; accepted 24 April 2015. © 2015 The Australasian College of Dermatologists
Key words: diagnostic criteria, erosive vulvovaginal lichen planus, genital lichen planus.
INTRODUCTION Lichen planus is a chronic inflammatory disorder affecting skin and mucous membranes including the vagina, vulva, oral cavity, ear canals, oesophagus and lacrimal ducts. Its overall prevalence is unknown but is estimated to be around 1%.1 The vulvovaginal form is rare and may occur in isolation or in combination with oral disease (the vulvovaginal gingival syndrome2) or other mucocutaneous disease. Vulvovaginal disease usually results in erosions leading to chronic vulval pain, difficulty with or complete cessation of sexual activity and discomfort on micturition and defecation. Other reported symptoms include itch, vaginal discharge and bleeding.3 The long-term risk of associated vulval carcinoma remains unclear.4 Adding to the burden of disease, response to treatment is often disappointing and the clinical course chronic, irrespective of therapy.5 A recent review of mucosal lichen planus by the Cochrane Collaboration found only weak evidence for the effectiveness of any of the treatments for oral disease and did not find any studies of genital disease.6 There have been several retrospective studies documenting clinical symptoms and signs in erosive vulvovaginal lichen planus (EVLP).2,3,7 Commonly reported clinical findings include sharply demarcated erythematous lesions or erosions, hyperkeratotic areas, Wickham striae (fine white lines over the surface of lesions) and labial fusion leading to introital stenosis (Fig. 1). A single prospective cohort study found vulval pain was reported in 80% and vulval erosions were present in 97%.8
Abbreviation: EVLP
erosive vulvovaginal lichen planus
2
H Cheng et al. Table 2 Frequency of symptoms and signs in women with erosive vulvovaginal lichen planus
Symptoms Pain or burning Itch Clinical signs Well-demarcated erosions or erythematous areas at the vaginal introitus Scarring or loss of architecture White plaques Hyperkeratotic border to lesions or Wickham striae Involvement of other mucosal sites Vaginal inflammation Papules
Figure 1 Characteristic clinical findings in erosive vulvovaginal lichen planus. Well-defined introital erosion and extensive architectural change with labial resorption, buried clitoris and introital stenosis. Table 1
Diagnostic criteria for erosive vulvovaginal lichen planus9
Well-demarcated erosions or erythematous areas at the vaginal introitus Presence of a hyperkeratotic border to lesions or Wickham striae in surrounding skin Symptoms of pain or burning Scarring or loss of normal architecture Presence of vaginal inflammation Involvement of other mucosal surfaces Presence of a well-defined inflammatory band involving the dermoepidermal junction Presence of an inflammatory band consisting predominantly of lymphocytes Signs of basal layer degeneration
Recently, clinicopathological diagnostic criteria have been suggested following an electronic Delphi consensus exercise involving experts in the diagnosis and management of vulval disease.9 Nine supportive diagnostic criteria reached the consensus (Table 1). It was suggested that at least three supportive features should be present to diagnose EVLP. The aim of this study was to review the clinical and histological features of EVLP in a cohort of women from New Zealand in particular, to assess the frequency of suggested diagnostic criteria.
MATERIALS AND METHODS New Zealand Health and Disability Ethics Committee review was not required, as this was a retrospective obser© 2015 The Australasian College of Dermatologists
Frequency (n = 72)
%
66 36
92 50
69
96
63 32 33
88 50 46
27 24 2
38 33 3
vational study with minimal risk. Women with a clinical diagnosis of EVLP who had attended specialist dermatology clinics at two sites in New Zealand from September 2002 to November 2013 were included. Cases where EVLP was not the primary skin disorder or where there was uncertainty of the diagnosis were excluded. Cases where clinical images were of poor quality were excluded. Written consent had been obtained for clinical photographs to be used in a research setting. Clinical signs were identified from photographs by a dermatologist with expertise in vulval skin disease. Symptoms and clinical signs that were not visible in photographs (e.g. vaginal involvement, other mucosal disease) were identified from clinical notes. Histological reports were reviewed where available.
RESULTS In total, 89 women were identified and of these 17 were excluded; nine due to uncertainty of EVLP as the primary diagnosis, five due to the poor quality of the clinical images and three due to insufficient clinical information. A total of 72 women were included in the final analysis with an average age of 67 years (range 26–93). Clinical photographs were available for all patients. Frequency of symptoms and signs are shown in Table 2. Accompanying disease at other mucosal sites was found in 36% of the participants. Most frequently this was oral (23/ 72) or anal (nine). Two women had oesophageal disease. Architectural changes included labial resorption (56), buried clitoris (21) and introital stenosis (18). A vulval biopsy was performed in 55/72 women (76%) and 45 histology reports were accessed. Histological findings included a band of inflammation with predominant lymphocytes (35/45), a well-defined inflammatory band at the dermoepidermal junction (32) and evidence of basal layer degeneration (27) (Fig. 2). Six cases (13%) displayed non-specific findings with none of the histological features suggested as diagnostic criteria by Simpson and colleagues. No other inflammatory disorder or neoplasia was reported in these biopsies.
Erosive vulvovaginal lichen planus Of the diagnostic criteria suggested by Simpson and colleagues, the most common were well-demarcated erosions or erythematous areas at the vaginal introitus (96%), symptoms of pain or burning (92%) and scarring or loss of architecture (88%). The median number of diagnostic criteria met was five. Overall, 70 patients met at least three of the nine diagnostic criteria (97%); 59 patients met at least four diagnostic criteria (82%) (Fig. 3).
DISCUSSION EVLP is a painful and chronic genital dermatosis. Women often present late, after months or years of symptoms or misdiagnosis. In our group, pain was by far the most commonly documented symptom. Of the cases where pain or burning was not documented, clinical notes often stated
Figure 2 Histological features of erosive vulvovaginal lichen planus. Inflammatory infiltrate with lymphocyte predominance at the dermoepidermal junction. Degeneration of the epidermal basal layer, arrow shows necrotic keratinocyte (H–E ×200).
3
discomfort or other vague descriptors. A number of studies have documented pain or soreness as a common presenting symptom in EVLP.3,7,8 A retrospective study of 95 women with EVLP in The Netherlands found the most common presenting symptoms to be dyspareunia (54%), vulval soreness or burning (50%) and pruritus (48%).3 Similar to our findings, sharply demarcated erythematous lesions were the most common clinical finding (81%). Overall, 114 women were included in a prospective cohort study in Oxford, England that examined clinical symptoms and signs of vulval lichen planus and response to treatment.8 Again, vulval pain and pruritus were the most common symptoms (80 and 65%, respectively). Vulval erosions were found in 97% of cases. Just over half of cases had vaginal involvement (58%). Clitoral burying and introital narrowing were common (68 and 59%, respectively). These findings, combined with our own suggest that welldemarcated red areas or erosions are the most common clinical finding in EVLP. However, these may not be specific. Erosions are areas with partial epidermal loss and are also found in lichen sclerosus, which is a much more common genital dermatosis. Symmetrical erosions at the introitus are suggestive of EVLP. Introital stenosis and loss of architecture are also features shared with lichen sclerosus. Lichen planus and lichen sclerosus have overlapping histological features, particularly in the early stages. A band of inflammation at the dermoepidermal junction can be found in both disorders. The additional finding of homogenisation of collagen in the superficial dermis is suggestive of lichen sclerosus. When it is difficult to differentiate between the two conditions, both clinically and histologically, a dual diagnosis can be considered. Over time, women with lichen sclerosus develop shiny, hypopigmented and sclerotic skin. Vaginal involvement is rare in lichen sclerosus but has been reported in association with severe disease and uterine prolapse.10 Vulval disease due to oestrogen deficiency, mucous membrane pemphigoid, fixed drug eruption, plasma cell vulvitis
Figure 3 Number of diagnostic criteria present in 72 women with erosive vulvovaginal lichen planus. © 2015 The Australasian College of Dermatologists
4
H Cheng et al.
and graft versus host disease may also be difficult to distinguish from EVLP on clinical grounds alone and diagnosis requires vigilance. These conditions often have distinctive findings on histological examination and immunofluorescence. The nine recently published diagnostic criteria for EVLP were the outcome of an electronic Delphi consensus exercise.9 It was suggested that at least three criteria should be met to be confident in a diagnosis. However, this number is up for debate; a number of experts considered four to be a more appropriate number, presumably to reduce false positives (particularly in women with lichen sclerosus). In all, 97% of the women in our study met at least three diagnostic criteria. The remaining two women met two criteria. Of these, one had red shiny areas at the introitus; however, they were poorly demarcated. The biopsy report for the second woman was unavailable for review. A diagnosis of EVLP may be made on clinical grounds alone, although a biopsy should be undertaken where there is diagnostic uncertainty or suspicion of neoplasia. A biopsy was performed in nearly three-quarters of the patients in our group. Of available reports, 84% of the biopsies met at least one of the histological criteria suggested. Histopathological findings in EVLP include epidermal ulceration with the typical features of lichen planus at the edge of the lesions (Fig. 2). The infiltrate often contains lymphocytes, although inflammation may be sparse in endstage disease. Plasma cells are also a frequent finding in genital sites. A lichenoid infiltrate with plasma cells is also found in plasma cell vulvitis. Red cell extravasation, dermal fibrosis and a relative abundance of plasma cells can help to differentiate plasma cell vulvitis from EVLP histologically.11 The main limitation of this study is its retrospective design. Patients who were included had a firm clinical diagnosis of EVLP and therefore we cannot comment on the usefulness of the diagnostic criteria in more difficult cases. Further studies are required to validate diagnostic criteria in real-life clinical setting, particularly in differentiating between EVLP and other vulval dermatoses. Another limitation is the lack of histology for some cases. Ideally, for the benefit of research, histological diagnosis should be available for all patients. However, previous studies have shown that histological findings in EVLP can be variable, including chronic inflammation, lichen sclerosus and other non-
© 2015 The Australasian College of Dermatologists
specific findings.3 Because of this we also included women with a clinical diagnosis of EVLP made by expert clinicians with many years of experience in vulval dermatology. In summary, symptoms of pain or burning, welldemarcated red shiny areas or erosions at the introitus and scarring or loss of architecture were the most common findings and were present in most of the women with erosive lichen planus. Our findings support the diagnostic criteria suggested by Simpson and colleagues. Most patients met at least three diagnostic criteria. A validation of the EVLP diagnostic criteria with prospective studies is required.
REFERENCES 1.
2.
3.
4. 5.
6.
7.
8.
9.
10.
11.
Eisen D. The clinical features, malignant potential, and systemic associations of oral lichen planus: a study of 723 patients. J. Am. Acad. Dermatol. 2002; 46: 207–14. Pelisse M, Leibowitch M, Sedel D et al. A new vulvovaginogingival syndrome. Plurimucous erosive lichen planus. Ann. Dermatol. Venereol. 1982; 109: 797–8. Santegoets L, Helmerhorst T, van der Meijden W. A retrospective study of 95 women with a clinical diagnosis of genital lichen planus. J. Low. Gen. Tract. Dis. 2010; 14: 323–8. Simpson R, Murphy R. Is vulval erosive lichen planus a premalignant condition? Arch. Dermatol. 2012; 148: 1314–6. Cribier B, Fraces C, Chosidow O. Treatment of lichen planus. An evidence based medicine analysis of efficacy. Arch. Dermatol. 1998; 134: 1521–30. Cheng S, Kirtschig G, Cooper S et al. Interventions for erosive lichen planus affecting mucosal sites. Cochrane Database Syst. Rev. 2012: CD008092. Bradford J, Fischer G. Management of vulvovaginal lichen planus: a new approach. J. Low. Genit. Tract Dis. 2012; 17: 28–32. Cooper S, Wojnarowska F. Influence of treatment of erosive lichen planus of the vulva on its prognosis. Arch. Dermatol. 2006; 142: 289–94. Simpson RC, Thomas KS, Leighton P et al. Diagnostic criteria for erosive lichen planus affecting the vulva: an international electronic-Delphi consensus exercise. Br. J. Dermatol. 2013; 169: 337–43. Kendell K, Edwards L. Lichen sclerosus with vaginal involvement: report of 2 cases and review of the literature. JAMA Dermatol. 2013; 149: 1199–202. Weedon D. Skin Pathology, 2nd edn. New York: Churchill Living stone, 2010.
