11/1/18. Age and Vulvar Pathology ANATOMY. Prepuce Clitoris Vestibule Hart line. Labia. minora. Labia. majora. Fourchette.

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1 Age and Vulvar Pathology Maria Angelica Selim, MD Professor of Pathology and Dermatology Director Dermatopathology Unit Duke University Medical Center Labia minora Labia majora Bartholin s duct Prepuce Clitoris Vestibule Hart line Fourchette ANATOMY 1

2 Fetal 1-10 years years years years years 2

3 James McCartney Skin deep The great wall of vagina Practical points Variation of normal histology with age and area of the vulva biopsied Morphology can be atypical, low threshold to biopsy and re-biopsy Superimposed processes are common Contact dermatitis from prescribed treatment (iatrogenic) Candida infection superimposed on contact dermatiits 3

4 + Vulvar Disease in 130 Children Diagnosis n % of total Dermatitis 41 33% Lichen sclerosus 23 18% Psoriasis 21 17% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D. THE INTERNATIONAL SOCIETY FOR THE STUDY OF VULVOVAGINAL DISEASE (ISSVD) 4

5 ISSVD Classification of Vulvar Dermatosis Spongiotic pattern Atopic dermatitis Allergic dermatitis Irritant contact dermatitis Acanthotic pattern Psoriasis Lichen simplex chronicus Primary Seconday Lichenoid pattern Lichen sclerosus Lichen planus Dermal homogenization /sclerosis pattern Lichen sclerosus Vesiculobullous pattern Pemphigoid cicatricial type Linear IgA disease Acantholytic pattern Hailey-Hailey disease Darier disease Papular genitocrural acantholysis Granulomatous pattern Crohn s disease Melkersson-Rosenthal syndrome Vasculopathic pattern Apthous ulcers Behcet disease Plasma cell vulvitis J Reprod Med Jan;52(1):3-9 Vulvar Disease in 130 Children Diagnosis n % of total Dermatitis 41 33% Lichen sclerosus 23 18% Psoriasis 21 17% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D. ISSVD Classification of Vulvar Dermatoses Spongiotic pattern Allergic dermatitis Irritant contact dermatitis Contact Dermatitis. 2004;51:

6 ü Compared with the adult vulva, the pediatric vulva is hairless and has a very little subcutaneous fat under the mons pubis and labia majora ü The labia minora have an atrophic appearance ü The vestibule is composed of squamous epithelium resembling vaginal mucosa, which is not glycogenated in the pediatric patient and does not have estrogen effect until puberty 2 year old with irritation and rawness. Mom washes the area three times a day with Ivory Soap, and uses adult wipes. Resistant to multiple anticandidal creams 6

7 Symptoms are irritation, burning, sometimes itching. Prepubertal genital skin is easily irritated Mothers over-wash and over-medicate little girls vulvas. Less is best In atopic children, irritants precipitate localized atopic dermatitis Allergic contact dermatitis is rare in childhood genital disease and is usually secondary to treatment for another, underlying condition. Doesn t matter stop everything except water and your medications PSEUDOWARTS Also called granuloma gluteale infantum, Jacquet s diaper dermatitis ü Unusual manifestation of irritant contact dermatitis seen almost exclusively in small children ü Flat-topped, skin colored to pink symmetrical papules, sometimes with overlying erosion ü Background of variable erythema 7

8 Vulvar pseudoverrucous papules/ nodules or Granuloma gluteale infantum Location: perianal, genital, suprapubic and peristomal areas and on the buttocks Age: inflants and adults Origin: irritant contact dermatitis Garrido-Ruiz Luis et al. Am J Dermatopathol 2011 Goldberg Neil et al. Arch Dermatol 1992;128:240-2 Vulvar Disease in 130 Children Diagnosis n % of total Dermatitis 41 33% Lichen sclerosus 23 18% Psoriasis 21 17% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D. Pediatric Dermatology 1-7,

9 Pediatric Dermatology 1-7, 2013 Vulvar Disease in 130 Children Diagnosis n % of total Dermatitis 41 33% Lichen sclerosus 23 18% Psoriasis 21 17% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D Vol17:1-6, J Low Genit Tract Dis Oct;18(4):e84-9. Pediatric Dermatology (3)

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11 BULLOUS PEMPHIGOID LOCALIZED TO VULVA 11

12 Vulvar Disease in 130 Children Diagnosis n % of total Dermatitis 41 33% Lichen sclerosus 23 18% Psoriasis 21 17% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D. Staphylococcal scalded skin syndrome 12

13 Childhood linear IgA disease Granulomas in Vulva Foreign body giant cell reaction Crohn s disease 13

14 Nonspecific Lesions Aphthous ulcers (mouth or vulva) Pyostomatitis vegetans (mouth) Erythema nodosum (legs) Pyoderma gangrenosum (usually legs) Cutaneous vasculitis (usually legs) Prepubertal Vulval Fibroma CD34 14

15 ISSVD Classification of Vulvar Dermatoses Spongiotic pattern Allergic dermatitis Irritant contact dermatitis Langerhan granuloma 15

16 Common Vulvar Contactants ALLERGENS Benzocaine (Vagisil) Preservatives Neomycin Latex condoms Chlorhexadine (KY) Lanolin Perfume Nail Polish IRRITANTS Soaps/cleansers Sweat, urine, feces Creams (alcohol) Douches Medications TCA, 5FU Spermicides Panty liners Vulvar Disease: 130 Children 144 adult Diagnosis n % of total Dermatitis 41 33% 54% Lichen sclerosus 23 18% 13% Psoriasis 21 17% 5% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D. Pediatric dermatol Vol17:1-6, The commonest causes of symptomatic vulvar disease: a dermatologist s perspective Gayle Fischer M.D. Australas J Dermatol (1):12 LICHEN SCLEROSUS 16

17 Clues for early diagnosis of LS Clues for differentiated VIN in LS ISSVD Classification of Vulvar Dermatosis PAS Elastic stain Fung et al. Am J Surg Pathol 1998;22:473-8 Regauer et al. Histopathology 2005;47:340-7 Psoriasiform lichenoid dermatitis Lymphocytes aligned in rows Epidermis peppered by lymphocytes. Thickening BM. Lymphocytes align the basal layer and dyskeratotic cells 17

18 LICHEN PLANUS Mucosal LP Cutaneous LP 18

19 Lichen Planus Scarring is prominent on vulva and in vagina Courtesy of Dr. Gordon Davis Br J Dermatol Jul;169(1): p53 p53 19

20 LICHENOID DRUG ERUPTION 20

21 ZOON S VULVITIS Plasma cells 0-25% of the infiltrate Nonspecific Related to mucosal site Plasma cells 25-50% of the infiltrate ü Diamond shape keratinocytes ü Red cell extravasation ü Iron stain Plasma cells > 50% of the infiltrate PLASMA CELL VULVITIS EMLA J Cutan Pathol Aug;40(8):

22 Lichenoid drug eruption Lichenoid inflammation Eosinophils Zoon s vulvitis Plasma cells /red cell extravasation Perl stain Syphilis EMLA Plasma cells/endothelialitis Spirochete IHC Lichen Planus (mucosa) Lichen Planus (cutaneous) Vulvar Disease: 130 Children 144 adult Diagnosis n % of total Dermatitis 41 33% 54% Lichen sclerosus 23 18% 13% Psoriasis 21 17% 5% Streptococcal vulvitis 13 10% Staphylococcal folliculitis 4 5% Labial fusion 3 4% Genital warts 2 <1% Bullous pemphigoid 2 <1% Tinea 2 <1% Scabies nodules 1 <1% Erythema annulare centrifigum1 <1% Vitiligo1 <1% Molluscum contagiosum1 <1% Vulvar Disease in Children: A Clinical Audit of 130 Cases Gayle Fischer M.D., Maureen Rogers M.D. Pediatric dermatol Vol17:1-6, The commonest causes of symptomatic vulvar disease: a dermatologist s perspective Gayle Fischer M.D. Australas J Dermatol (1):12 22

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25 Bovine collagen Hyaluronic acid Silicone Paraffin J Am Acad Dermatol 2011;64:

26 VULVAR CANCER 3 to 4% of genital female cancers 90% Squamous Cell Carcinomas Two types of pathways Judson PL et al, Obstet Gynecol 2006 De Koning MNC et al, Mod Path

27 A 14 years Dutch survey ( ) HSIL (VIN, usual type) VIN, differentiated type months 23 months 5% SCC 35% Van de Nieuwenhof et al, Eur J Cancer, 2009 TERMINOLOGY AND CLASSIFICATIONS ISSVD 1986 ISSVD 2004 LAST 2012 WHO 2014 ISSVD 2015 Vulva Intraepithelial Neoplasm (VIN) Vulva Intraepithelial Neoplasm Vulva Intraepithelial (VIN) Lesion Vulva Intraepithelial Lesion Vulva Intraepithelial Lesion 1. Squamous type (with Flat condyloma or HPV effect LSIL LSIL or w/o HPV change) a) VIN I LSIL (Flat condyloma or HPV effect) a) VIN II b) VIN III (Formely: SCCIS, Erythropesia of Queyralt, CIS simplex) VIN, usual type HSIL HSIL HSIL (uvin) - VIN, warty type - VIN basaloid type - VIN mixed (warty/basaloid) type VIN, differentialted type (dvin) VIN, differentialted type (dvin) VIN, differentialted type (dvin) 27

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30 Modern Pathology (2013) 26,

31 Ki-67 p53 Acta Oncol. 2004;43(5):

32 NORMAL LS uvin dvin dvin Loos 25-75% Loss >75% 32

33 P53 33

34 77-year-old patient with a right deep hemi-vulvectomy for a pt2 SCC at age 70 years and one recurrence at age 75 years 54 months 6 months Atypical LS, squamous hyperplasia with atypia, atypical hyperplastic dystrophy A wide variation of exophytic, verrucous, flat and atrophic HPV-negative squamous intraepithelial lesions/precursor lesions different from d-vin, which are not addressed in the present classification systems of VIN. 34

35 - Percentage of dvin lesions with subsequent diagnosis of SCC: 32.8% - Median time for progression from dvin to SCC: 23 months 35

36 Thank you 36