Pattern of non-venereal dermatoses of female external genitalia in South India
Published Web Locationhttps://doi.org/10.5070/D35hs3f52v
Pattern of non-venereal dermatoses of female external genitalia in South India1. Department of Dermatology and Sexually Transmitted Diseases, Jawaharlal Institute of Postgraduate Medical Education and
Research (JIPMER), Pondicherry-605006, India. firstname.lastname@example.org; email@example.com
Nidhi Singh MBBS1, Devinder Mohan Thappa MD DHA MNAMS1, TJ Jaisankar MD1, Syed Habeebullah, MD2
Dermatology Online Journal 14 (1): 1
2. Department of Obstetrics and Gynaecology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry-605006, India
Non-venereal dermatoses tend to be confused with venereal diseases, which may be responsible for mental distress and guilt feelings in patients. We conducted the study to find the pattern of non-venereal dermatoses of female external genitalia and to correlate non-venereal dermatoses with various clinical parameters. The study included 120 female patients with non-venereal dermatoses of female external genitalia presenting over a period of 22 months from September 2005 to June 2007. The demographic characteristics and clinical findings were recorded. Cases having venereal diseases were excluded from the study. A total of nineteen non-venereal dermatoses were noted in the study. The most common non-venereal dermatoses were lichen sclerosus (26 cases or 21.7%), vitiligo (19 cases or 15.8%), lichen simplex chronicus (16 cases or 13.3%), and vulval candidiasis (11 or 9.2%). Other dermatoses included lymphedema, invasive squamous cell carcinoma, tinea cruris, psoriasis, furuncle, folliculitis, lichen planus, epidermal inclusion cyst, herpes zoster, irritant contact dermatitis, acrochordon, Bartholin cyst, fibroepithelial stromal polyp, molluscum contagiosum (autoinoculated), and streptococcal vulvitis. This study highlights the importance of diagnosing non-venereal dermatoses and refutes the general misconception that all vulval itching is the result of fungal infection. The two most common causes of vulval itching observed in the study were lichen sclerosus and lichen simplex chronicus.
Dermatoses involving female external genitalia are not always sexually transmitted. Those which are not sexually transmitted are referred to as non- venereal dermatoses of female external genitalia . Recently vulvar dermatoses have been classified by the International Society for the Study of Vulvovaginal Disease (ISSVD) inclusive of venereal and non-venereal vulvar dermatoses . However, non-venereal dermatoses of female genitalia are not strictly classified . They include inflammatory cutaneous disorders (psoriasis, seborrheic dermatitis, lichen planus, lichen sclerosus), autoimmune (vitiligo), multisystem diseases (Behcet syndrome, Reiter syndrome, Crohn disease), exogenous (contact dermatitis, corticosteroid abuse, fixed drug eruption), and benign and malignant neoplasms (extramammary Paget disease) [1, 3].
Because venereal and non-venereal dermatoses tend to be confused, the occurrence of these dermatoses may be associated with mental distress and guilt feelings in affected patients. Although the literature is saturated with case reports of non-venereal dermatoses, no formal study has been done on the overall occurrence. Hence, we undertook this study to find the pattern of non-venereal dermatoses in female external genitalia and their relative frequencies at a tertiary care centre in south India.
This is a descriptive study spanning a period of 22 months from September 2005 to June 2007. All female patients attending the Skin outpatient department (OPD) and Obstetrics and Gynecology OPD at Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, were screened for non-venereal dermatoses of female external genitalia. The Institute ethics committee clearance was obtained. All consenting female patients irrespective of their age and pregnancy status who presented with genital complaints were screened for non-venereal dermatoses. Informed consent was obtained. A detailed history including demographic data, chief complaints related to skin, presence of itching, skin lesions, onset, pregnancy status, menstrual status, and associated medical or skin disorders was elicited and recorded. Enquiry was made with regard to history of sexual exposure. Cases having venereal diseases were excluded from the study.
The external genitalia were examined and findings were noted. A detailed physical examination was made to see any associated lesions elsewhere in the body. Investigations such as Gram stain and KOH mount were done as and when required to establish the diagnosis. Biopsy and histopathological examination of the specimen was done when required to confirm the diagnosis. Results were tabulated and analyzed using SPSS 13.0 software.
A total of 120 female patients with non-venereal dermatoses of external genitalia were included in this study. The age of the patients ranged from one year to 85 years, with a mean age of 37 years. Most patients belonged to the age group of 41-60 years (50 patients, 41.6%), followed by the age group 31-40 years (21 patients, 17.5%). Eighty-two patients (68.3%) were from rural areas and 38 patients (31.7%) were inhabitants of urban areas. Ninety-eight (81.6%) of them were married, and the remaining 22 (18.3%) patients were unmarried. Most of the women were in the postmenopausal age group (50 patients, 41.7%) closely followed by women in the reproductive age group (49 patients, 40.8%). Young girls who had not attained menarche accounted for 21 (17.5%) patients. Seven women (5.8%) were pregnant. The majority of these cases were laborers (37.5%), followed by housewives (35.8%), students (12.5%) and preschool children (5%).
The common presenting feature was itchy genitalia (61.7% of cases) followed by white discoloration (27.5%) and swelling (12.5%). Other complaints were pain, burning sensation, mass, dyspareunia, redness, exfoliation of skin, raised lesions over skin, oozing, constipation, burning micturition, ulceration, erosion and thickening of skin. Some patients had more than one complaint.
The labia majora was the most common site of involvement, accounting for 110 (91.7%) cases followed by labia minora in 58 (48.3%) cases, clitoris in 32 (26.7%) cases, mons pubis in 16 (13.3%) cases, and introitus in three (2.5%) cases.
A total of 19 different types of non-venereal dermatoses were noted in our study. The most common non-venereal dermatosis was lichen sclerosus (Figs. 1 and 2) which constituted 26 (21.7%) cases followed by 19 (15.8%) cases of vitiligo (Fig. 3); 16 (13.3%) cases of lichen simplex chronicus (Figs. 4 and 5); 11 (9.2%) cases of vulval candidiasis (Fig. 6) and others (Figs. 7 through 13).
The different types of non-venereal dermatoses of female external genitalia seen in our study can be grouped into:
A. Inflammatory disorder (59 patients)
- Lichen sclerosus 26
- Lichen simplex chronicus 16
- lymphedema 7
- Psoriasis 5
- Lichen planus 3
- Irritant contact dermatitis 2
B. Pigmentary disorders (19 patients)
- Vitiligo 19
C. Non venereal infections and infestations (28 patients)
- Candidiasis 11
- Tinea cruris 6
- Furuncle 4
- Folliculitis 3
- Herpes zoster 2
- Molluscum contagiosum (autoinoculated) 1
- Streptococcal vulvitis 1
D. Benign tumors and cysts (8 patients)
- Epidermal inclusion cyst 3
- Acrochordon 2
- Bartholin cyst 2
- Fibroepithelial stromal polyp 1
E. Malignant tumors (6 patients)
- Invasive squamous cell carcinoma 6
|Figure 1||Figure 2|
|Figure 1. Late stage lesions of lichen sclerosus (LS) with telangiectasia and erosions, atrophy of labia minora, burying of
clitoris and introital narrowing.|
Figure 2. "Figure of 8" appearance in LS. (CLICK for full size images)
|Figure 3||Figure 4|
|Figure 3. Depigmented macule over genitalia with trichrome sign - Vitiligo.|
Figure 4. Symmetrical lichenified plaques over labia majora - Lichen simplex chronicus.
|Figure 5||Figure 6|
|Figure 5. Lichen simplex chronicus leading on to hypertrophy of labia minora.|
Figure 6. Bright red erythema topped by white deposits in periorificial area - Vulval candidiasis.
|Figure 7||Figure 8|
|Figure 7. Massive vulval lymphedema resulting in esthiomene-like appearance.|
Figure 8. Chronic vulval lymphedema with lymphangiectasia and lymphorrhea over labia majora.
|Figure 11||Figure 12|
|Figure 11. Pultaceous material expressed out from a cyst - Epidermal inclusion cyst.|
Figure 12. Herpes zoster involving L1, L4 and S3 dermatomes with genital involvement.
|Figure 13: Skin tag over left labium majus - Acrochordon.|
The most common non-venereal dermatoses in prepubertal girls (21 cases) were vitiligo (12 patients, 57.1%), lichen sclerosus (three patients, 14.3%) and psoriasis (two patients, 9.5%); in the reproductive age group (49 cases) were vulval candidiasis (10 patients, 20.4%), lichen simplex chronicus (six patients, 12.2%), lichen sclerosus (five patients, 10.2%) and tinea cruris (five patients, 10.2%); and in postmenopausal women (50 cases) were lichen sclerosus (18 patients, 36%), lichen simplex chronicus (10 patients, 20%), invasive squamous cell carcinoma (six patients, 12%), lymphedema (five patients, 10%) and vitiligo (four patients, 8%). Vulval candidiasis was the most common non-venereal dermatosis in pregnant women accounting for six cases out of the seven pregnant patients. There was no correlation observed between occupation and non-venereal dermatoses.
Some patients of non-venereal dermatoses also had other associated disorders. Diabetes mellitus was present in two cases each of candidiasis and lichen simplex chronicus. Five patients who had received radiotherapy for carcinoma cervix presented with vulval lymphedema. Tuberculous lymphadenitis and filariasis was associated with one case each of vulval lymphedema.
Lichen sclerosus (LS) was observed in 26 cases with a mean age of 44 years. The most common presenting complaint was itching (24 cases), followed by color change (15 cases), and dyspareunia (2 cases). Duration of complaints ranged from two weeks to 40 years, with a median value of 2.5 years.
Labia majora along with labia minora and clitoris was the most commonly involved site in lichen sclerosus accounting for 15 (57.7%) patients. Next common site of involvement was labia majora along with labia minora in seven (26.9%) patients. Perianal involvement was present in eight (30.8%) patients. All patients presented with ivory white atrophic plaques. Surface of the plaque showed telangiectasia in one patient; both erosions and fissuring in two patients, erosions in four patients and fissuring in two patients and wrinkling in all patients. Introitus was stenosed in five (19.2%) patients, out of which three (11.5%) patients also had perianal involvement leading to "figure of 8" appearance. Three patients had atrophy of labia minora and clitoris to an extent that labia minora appeared merged with labia majora and clitoris was buried.
Two patients of LS were also found to have vitiligo, one had acrofacial vitiligo and the other had focal vitiligo.
Vitiligo was observed in 19 cases with a mean age of 19.6 years. All patients came with asymptomatic white discoloration over genitalia. Duration of complaints ranged from 10 days to 15 years with a median period of 18 months.
All patients had depigmented macules. Trichrome sign was present in nine patients and one patient had leukotrichia. Labia minora and labia majora were involved in 16 out of 19 cases each, irrespective of whether they were the only site involved or are involved in combination with other parts of vulva. There were 12 cases of focal genital vitiligo, 5 cases of vitiligo vulgaris, and one case each was of acral and mixed vitiligo.
Lichen simplex chronicus
Lichen simplex chronicus (LSC) was observed in 16 cases with a mean age of 49.9 years. All of them complained of intractable itching, the duration of which varied from 1 year to 20 years with a median duration of 2 years. Lichenification was found in all the cases and the most common site involved was labia majora alone accounting for 14 of 16 cases and only in the remaining two cases there was involvement of labia minora also along with labia majora, and out of these two cases, one had hypertrophy of labia minora and other had clitoral hypertrophy. Thus, labia majora was involved in all cases of lichen simplex chronicus. All the cases had bilaterally symmetrical involvement except one case having asymmetrical involvement on the side of the dominant hand (right side). All our cases of LSC were primary. All the patients of lichen simplex chronicus admitted to having mental stress.
Non-venereal dermatoses of female external genitalia include a spectrum of diseases with varied etiology. Genital diseases may be associated with severe psychological trauma and fear in the mind of patients. Therefore, it is of immense importance to diagnose these non-venereal dermatoses to relieve the patient from the stigma of sexually-transmitted diseases. There are no comprehensive studies on the pattern of non-venereal dermatoses from a developing country like ours. The most common non-venereal dermatoses observed in our study were lichen sclerosus (26 patients, 21.7%), vitiligo (19 patients, 15.8%), lichen simplex chronicus (16 patients, 13.3%) and vulval candidiasis (11 patients, 9.2%). The most common causes of vulvar itching were lichen sclerosus and lichen simplex chronicus. In the Sullivan et al.  study, the most frequent initial clinical diagnoses in a multidisciplinary vulvar clinic were lichen sclerosus (35, 26%), vaginal candidiasis (21, 16%), vulvodynia (16, 12%), lichen simplex chronicus (13, 10%), and Bowenoid papulosis (13, 10%). In a similar retrospective study of the referral patterns to a specialist vulval clinic reported by Cheung et al. , a total of 200 clinical records were reviewed of new patients seen between January 2004 and June 2005 and the most common condition seen was lichen sclerosus (39%), followed by eczema/lichen simplex (30.5%), lichen planus (11.5%), pain syndromes (10.5%) and others (8.5%). Fischer and Rogers  evaluated 130 prepubertal girls presenting to the dermatologist with a vulvar complaint to determine the spectrum of and frequency of conditions seen in this age group over a 3 year period. Of these patients, 41 (33%) had atopic or irritant dermatitis, 23 (18%) had lichen sclerosus, 21 (17%) had psoriasis, 15 (12%) had vulvar lesions (most often hemangiomas and nevi), and 13 (10%) had streptococcal vulvovaginitis. In our study, the most common non-venereal dermatosis in prepubertal girls (21 cases) was vitiligo (12 patients, 57.1%), followed by lichen sclerosus (three patients, 14.3%) and psoriasis (two patients, 9.5%). Vitiligo was recorded in a single case in a series of 130 cases by Fischer and Rogers , whereas lichen sclerosus was the second most common vulvar dermatosis as in our series. It is significant to note that vulvovaginal candidiasis, which represents a significant portion of adult vulvar disease, was not recorded by us as well as by Fischer and Rogers  in prepubertal girls.
Lichen sclerosus (LS) is chronic inflammatory dermatoses associated with substantial discomfort and morbidity . Anogenital LS is characterized by porcelain white atrophic plaques that may become confluent extending around vulval and perianal skin in a figure-of-eight configuration . The resulting atrophic plaque may have a cellophane-paper-like texture, wrinkled and fragile surface associated with telangiectasia, purpura, erosions, fissuring or ulceration [1, 8]. Clinical findings in cases of LS in this study were found to be in concordance with literature review. Atrophy can lead to loss of labia minora, burying of the clitoris, obstruction of urinary outflow, or other architectural changes . Architectural changes, such as atrophy of labia minora and clitoris, were observed in our study. The lesions occur in the inner aspects of labia majora, labia minora and clitoris . Perianal lesions occur in 30 percent of cases . This was consistent with our study where we found 30.8 percent patients having perianal lesions. Genital mucosal involvement does not occur in LS; the vagina and cervix are always spared. However, some mucosal involvement at the edge of mucocutaneous junctions may lead to introital narrowing . Introital narrowing was present in 19.2 percent patients of lichen sclerosus in our study. Involvement of labial, perineal and perianal areas along with introital narrowing is referred to as keyhole or hourglass or figure of 8 [3, 10]. Figure-of-8 appearance was present in three of our cases.
Although most studies have comprised Caucasian patients, there are reports of lichen sclerosus in Africans and Asians. Epidemiological surveys based on hospital referrals are likely to underestimate the prevalence of lichen sclerosus; estimates range from one in 300 to one in 1000 of all patients referred to dermatology departments . Powell et al.  calculated a prevalence of 1 in 660 women and an annual incidence rate of 51.9/100,000 in postmenopausal women. One study of pediatric vulvar LS reported a prevalence of 1:900, with a mean age at diagnosis of 6.7 years . The onset of LS has been reported at all ages although it is not common in less than 2 years of age . Lichen sclerosis has two peak ages of presentation: prepubertal girls and postmenopausal women (mean age of onset is fifth or sixth decade) . But in our study, we observed only one peak (postmenopausal) of presentation. In our study, the mean age of patients was 44 years (range 3-65 years); the median duration of symptoms of lichen sclerosus was 2.5 years (range 2 weeks-40 years) and the mean age at onset of symptoms of lichen sclerosus was 39.15 years (range 3-59.8 years). A study of 350 cases of women with lichen sclerosus by Thomas et al.  showed that the mean age of patients was 56 years (range 4-91 years); the mean duration of symptoms of lichen sclerosus was 10.5 years (range 1-65 years); and the mean age at onset of symptoms of lichen sclerosus was 45.5 years (range 1-90 years).
LS most commonly affects anogenital region (85-98%). Extragenital LS can be seen in 15-20 percent cases . Women complain of intractable pruritus (worse at night) [10, 15], irritation, soreness, dyspareunia, dysuria , and urinary or fecal incontinence [7, 16]. Nine percent of cases may be asymptomatic . Prepubertal girls usually complain of itching and soreness as in adults but they can also have dysuria, constipation , pain on defecation, soiling, fissuring, and bleeding [7, 18, 19]. Our observation was in concordance with literature except for the observation of only one peak (postmenopausal) of presentation contrary to the two peaks (prepubertal and postmenopausal) noted in literature.
Lichen simplex chronicus (LSC) may be primary (arising from normal appearing skin) or secondary (superimposed on other underlying disease) . All our cases of LSC were primary. Anogenital LSC is a common disease. Incidence and prevalence figures have not been established, but Lynch  estimate that it occurs in about 0.5 percent of the Western European and American population. In clinics solely devoted to the care of patients with vulvar disorders, LSC accounts for 10-35 percent of patients seen [4, 21, 22]. Anogenital LSC predominantly occurs in mid- to late-adult life . In our study, we found that the mean age of patients was 49.9 years. The duration of symptoms varied widely from one year to 20 years with a median duration of two years in our patients. Lichenification may sometimes be very severe leading to marked clitoral or labial hypertrophy . LSC is usually bilateral but sometimes it may be asymmetrical or unilateral determined by the dominant hand . Our study also revealed this characteristic finding as all cases had bilaterally symmetrical involvement except one case having asymmetrical involvement with lichenification only on the side of the dominant hand (right side). The most common site involved is labia majora but labia minora, vulvar vestibule and mons pubis can also be involved occasionally . Lichenification was present over labia majora in all our cases and two cases also had involvement of labia minora, and out of these two cases, one case also had clitoral hypertrophy.
Vitiligo is an acquired pigmentary disorder characterized by loss of melanocytes resulting in depigmentation . Approximately 0.1 percent to 4 percent of people worldwide are affected by vitiligo. Indian studies report 0.46 percent to 8.8 percent prevalence of vitiligo . Out of the 19 vitiligo cases in our study, 12 had focal genital vitiligo, 5 had vitiligo vulgaris, one case had acrofacial vitiligo and one had mixed vitiligo with genital involvement.
Lymphedema is swelling attributed to accumulation of lymph in tissue [26, 27]. It is associated with inadequate lymphatic drainage. If it is from intrinsic abnormality of lymph conducting pathways, then it is called primary lymphedema. Primary lymphedema usually involves the lower extremities. But if inadequate lymphatic drainage is from an acquired obstruction or obliteration of lymphatic channels, it is said to be secondary lymphedema. The common causes of secondary lymphedema are trauma (surgery, radiotherapy), infection (filariasis, tuberculosis), inflammation, and malignancy [26, 27]. We had seven cases of lymphedema, and all of them were secondary lymphedema. Five patients with ages ranging from 45 to 60 years had lymphedema secondary to radiotherapy-induced lymphatic damage and in all the five patients radiotherapy was being given for carcinoma cervix. The other two cases were secondary to tuberculous lymphadenitis and filariasis.
Vulvar carcinoma is relatively rare with an incidence of 1-2/100,000 women per year [28, 29]. The majority are squamous cell carcinomas, but verrucous carcinoma, basal cell carcinoma, melanoma and rare appendageal tumors are well recognized. Squamous cell carcinoma (SCC) of vulva is predominantly a disease of postmenopausal women [8, 30, 31]. The mean age at diagnosis is 65 years. The incidence of SCC increases with age, the risk of malignancy increasing to 10 fold above 75 years [31, 32]. In our study, all six patients of invasive squamous cell carcinoma were postmenopausal with mean age of 55.8 years (range 50- 65 years).
Epidermal inclusion cysts are most common small cystic tumors of the vulva . They are true cysts. Cysts consist of a cyst wall lined by squamous epithelium and a cyst cavity filled with keratinized debris. Because of the cheesy appearance of the contents it is commonly misinterpreted as sebaceous cyst. Epidermal inclusion cyst may be related to trauma. Viable stratified squamous epithelium, if buried beneath either skin or mucosa, may proliferate, secrete, and desquamate to form an inclusion cyst . Over the female genitalia epidermal inclusion cysts are commonly seen over the labia majora. They are usually multiple and asymptomatic . In our study, we observed three cases of epidermal inclusion cysts; two cases presented with solitary cyst and one with multiple cyst.
Vulval lichen planus (LP) usually presents as violaceous or erythematous papules or annular plaques or erosions with or without a lacy white border . These lesions may ulcerate. If only vulval involvement is present, then disease is more likely to be erosive, with most lesions around labia minora, clitoris and clitoral hood. Other clinical forms of vulval LP include pigmented flexural LP, Vulvovaginal-gingival lichen planus (VVG-LP) and lichen planopilaris. Pigmented flexural LP usually presents as brown pigmented patches over mons pubis, inguinal and genitocrural folds. It is also seen in inframammary areas and axilla . Lichen planus was observed in three patients in our study.
Contact dermatitis over vulva could be either irritant or allergic. Both conditions may be characterized by variable pruritus. Patients with irritant dermatitis tend to have exacerbations after washing whereas those with allergic contact dermatitis are more likely to report marked flares and remissions . Irritant contact dermatitis usually occurs in females who excessively wash, use douches or scented feminine hygiene products, or wear tight clothing, or pads [23, 35, 36]. Patients complain of itching or burning or both that is exacerbated by moisture or hot weather. Patients' efforts to reduce symptoms, such as frequent washing, scrubbing, and topical applications (home remedies or over the counter ointments), further exacerbate the dermatitis. Continued use of topical medications, home remedies, soaps and frequent washing leads to chronic irritant dermatitis [23, 35, 36, 37]. Irritant contact dermatitis to an antiseptic solution of chlorhexidine and cetrimide was observed in two cases in our study.
Acrochordon is a pedunculated fibroepithelial polyp commonly seen in intertriginous areas [33, 38]. These polyps may also be seen on labia majora and labia minora. Acrochordon is skin colored and soft projection of skin fold with a papillomatous or wrinkled surface. It can be single or multiple with size varying from a few millimeters to one cm [33, 38]. We observed acrochordon in two obese women.
Bartholin cysts are the most common cystic growths of the vulva . Two percent women develop Bartholin duct cyst or gland abscess at sometime in life. Bartholin gland abscesses are almost three times more common than Bartholin duct cysts . Gradual involution of the Bartholin glands occurs by 30 years of age. This is probably responsible for the more frequent occurrence of Bartholin duct cysts and gland abscesses during the reproductive years, especially between 20 and 29 years of age . It may start as an asymptomatic unilateral nontender cystic swelling, but it can cause pain and limitation of activity with increase in size [33, 40]. We observed two cases of Bartholin cyst.
Fibroepithelial stromal polyps are unusual benign lesions of vagina, which are rarely found over vulva, endometrium, cervix, nose, oral cavity, gastrointestinal and genitourinary tracts . These are benign mesodermal stromal polyps which can occur at any age. Fibroepithelial polyps consist of polypoid folds of connective tissue, capillaries, and stroma covered by epithelium of the site where it appears . We observed a single case of fibroepithelial polyp.
Streptococcal vulvitis is seen only in prepubertal girls . It is caused by group-A hemolytic Streptococci. It is thought that the infection spreads from pharyngeal infection, but clinical signs of throat infection are not always present. Streptococcal vulvovaginitis may precede or supervene upon vulval psoriasis. It presents with sudden onset erythematous, swollen, painful vulva and vagina, with a thin mucoid discharge. It may also present as subacute vulvitis. Vaginal and perianal swabs demonstrate the organism . We observed one case of streptococcal vulvitis.
Molluscum contagiosum is very common on vulva of girls . But it is usually a part of extensive eruption. It is unusual to have isolated involvement of vulva. In adults, molluscum over vulva is a sexually transmitted disease . We observed one case of molluscum contagiosum in an eight year old girl.
In conclusion, this study highlights the importance of diagnosing non-venereal dermatoses and refutes the general misconception of considering all vulval itching to be caused by fungal infection. The two most common causes of vulval itching observed in this study were lichen sclerosus and lichen simplex chronicus. Thus clinicians need to differentiate the various causes of non-venereal dermatoses.
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