2 dr mario sideri vv

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2 dr mario sideri vv

  1. 1. © IEO 2012 “ Vulavr and vaginal diseaes” Preti E*, Sideri M* *Preventive Gynecology Unit
  2. 2. © IEO 2012 Anatomical consideration Vulval skin comprises stratified squamous epithelium as in other parts of body The mons pubis and labia majora contain fat, sebaceous, apocrine and eccrine sweat glands and blood vessels , which can develop varicosities Labia minora are rich in sebaceous gland, contain few sweat gland but no hair follicles The epithelium of the vestibule is neither pigmented nor keratinized, but contain eccrine glands. These glands and epithelial appendages are a source of lump
  3. 3. © IEO 2012 Infection vulvar lesions • Herpes simplex/zoster • HPV • Chancroid, Lymphogranuloma venereum, syphylis • Bacterial infection • Amoebiasis • Epstein Barr virus • Histoplasmosis, tubercolosis actinomicesis
  4. 4. © IEO 2012
  5. 5. © IEO 2012 Benign Vulval lumps • Bartholin’s cyst. • Epidermal inclusion cyst. • Skene’s duct cyst. • Congenital mucous cysts: arise from mesonephric ducts remnante. • Cyst of the canal of Nuck: can give rise to hydrocele in labia maqjora. • Sebaceous cyst. • Papillomatosis (solid). • Fibroma (solid). • Lipoma (solid).  Cysts are either congenital or arise from obstructed glands.  Manifestations arise from the cysts (cosmetic) or from infection.
  6. 6. © IEO 2012 Case Report 2007 Lipoma (solid) S. M 30 years old 2006 Pain and edema of left labia majora 2006 Incision of the Bartholin’s cyst - - biopsy - - > adipose tissue
  7. 7. © IEO 2012 Non-neoplastic epithelial disorders • Lichen sclerosis. • Lichen planus. • Other common inflammatory dermatoses (lichen simplex, vulvar eczema, vulvar psoriasis) • Squamous cell hyperplasia (formerly: hyperplastic dystrophy). • Ulcerative dermatoses ( pemphigus , bullous pemphigoid, Hailey- Hailey disease, apthous ulceration, chron’s disease, Behcets disease) • Seborrhoeic dermatitis
  8. 8. © IEO 2012 The diagnosis of vulvar diseases presents an enigma. • For many years there was no classification of these diseases • Various clinical specialists (gynecologists, dermatologists, proctologists,and gastroenterologists) dealing with vulvar lesions • Benign vulvar lesions are various and correlate with dermatological – inflammatory – infection diseases
  9. 9. © IEO 2012 WHITE LESIONS leukoplakia kraurosis vulvar dystrophies chronic atrophic vulvitis hypertrophic vulvitis vulvar itthyosis lichen sclerosus Squamous intraepithelial lesions Intraepithelial carcinoma Bowen's Disease Carcinoma in situ vulvar atypia Erythroplasia Queyrat dysplasia bowenoid Vulvar intraepithelial neoplasia The beginnings of vulvar pathology …
  10. 10. © IEO 2012 2006 Classification of dermatologic vulvar disease ISSVD Spongiotic pattern a)atopic dermatitis b) Allergic contact dermatitis c) Irritant contact dermatitis Acantotic Pattern (previous benign squamous-cell) a) Psoriasi b) Lichen simplex cronicus Lichenoid Pattern a) Lichen sclerosus b) Lchen planus Sclerotic Pattern a) Lichen sclerosus b) Post-radiotherapy Vescicous- bullous Pattern a) Pemfigoidus b) Linear IgA Disease Acantolitic Pattern a) Hailey-Hailey Disease b) Darier Disease c) Papular genitocrural acantholysis Granulomatous Pattern Vasculopathic Pattern a) Chron Disease a) Afthous ulcer b) Melkenson-Rosenthal b) Bechet Disease c) Plasma cell vulvitis
  11. 11. © IEO 2012 International Society for the Study of Vulvar Disease (ISSVD) 1970 The founding of ISSVD helped to establish an ongoing dialog between representatives of the different disciplines in an attempt to arrive at an accepted nomenclature for diagnostic and therapeutic procedures - - - > multidisciplinary approach The first task was the systematic organization of the terminology of "white lesions"
  12. 12. © IEO 2012 12
  13. 13. © IEO 2012 13 Step 1 Define the lesion by choosing 1 or more of the following nouns (Blister – Bulla – Cyst – Edema – erosion – Excoriation – Fissure – Lesion – Macule – nodule – Papule – Plaque – Patch – Pustule – Rash – Ulcer – Vesicle) Step 2 Define color, margination , surface, configuration Step 3 Formulate a list of differential diagnoses (place an unrecognized vulvar disease into 1 of the 8 disease groups) Step 4 Reduce the number of diagnoses in the list of differential diagnoses (reading the brief sections on clinical morphology) Step 5 Confirm a clinical diagnosis (laboratory testing, biopsy ) DEFINITIONS, DESCRIPTION, AND DIAGNOSIS OF VULVAR LESIONS
  14. 14. © IEO 2012 14 2011 ISSVD Clinical Classification of Vulvar Dermatological Disorders
  15. 15. © IEO 2012 Complex classification Based on both the pathophysiology and the clinical manifestation of the disease High average experience and knowledge of vulvar pathology Does not include colposcopic pattern
  16. 16. © IEO 2012 The first doctor who deals with vulvar lesions is often the gynecologist colposcopist - Expert in diseases of the female genital tract - Colposcopic knowledge is not fully comparable to the vulvar pathology
  17. 17. © IEO 2012
  18. 18. © IEO 2012 18
  19. 19. © IEO 2012 19 Terminology for colposcopists and not expert clinicians Introduce basic and normal finding Educate and assist not expert clinicans in the diagnosis Include colposcopic pattern
  20. 20. © IEO 2012 20
  21. 21. © IEO 2012 Normality and anormality coexist
  22. 22. © IEO 2012 BASIC DEFINITION 22 Urethra Skene duct openings Pubis Labia majora, l. minora Vestibule Vestibular duct openings Bartholin duct openings Hymen Fourchette Perineum Anus Anal squamocolumnar junction (dentate line) Vestibolo Imene
  23. 23. © IEO 2012 BASIC DEFINITION 23 • Composition: Squamous epithelium: hairy/nonhairy, mucosa •Clitoris •Prepuce •Frenulum
  24. 24. © IEO 2012 NORMAL FINDINGS 24 Micropapillomatosis
  25. 25. © IEO 2012 NORMAL FINDINGS 25 sebaceous glands (Fordyce spots)
  26. 26. © IEO 2012 NORMAL FINDINGS 26 Vestibular redness
  27. 27. © IEO 2012 27 ABNORMAL FINDINGS Lesion type • Macule • Patch • Papule • Plaque • Nodule • Cyst • Vesicle • Bulla • Pustule
  28. 28. © IEO 2012 28 Definition sof prymary lesion types MACULE Small (<1.5 cm) area of color change, no elevation and no substance on palpation PATCH Large (>1.5 cm) area of color change, no elevation and no substance on palpation PAPULE Small (<1.5 cm) elevated and palpable lesion PLAQUE Large(>1.5cm) elevated, palpable, and flap topped lesion NODULE A large papule (>1.5 cm) often hemisherical or poorly marginated; may be located on surface, within, or below the skin; nodules may be cystic or solid VESICLE Small fluid-filled lister; (<0.5 cm) the fluid is clear (blister: a compartmentalized, fluid-filled elevation of the skin or mucosa) BULLA A large fluid-filled blister; the fluid is clear (> 0.5 cm) PUSTULE Pus-filled blister; the fluid is white or yellow Miscellanee Traumi – malformazioni
  29. 29. © IEO 2012 29
  30. 30. © IEO 2012 30
  31. 31. © IEO 2012 31 Each prymary lesion must be characterized for - Size - Location - Color - Secondary morphology ABNORMAL FINDINGS
  32. 32. © IEO 2012 32
  33. 33. © IEO 2012 33 ABNORMAL FINDINGS Secondary morphology
  34. 34. © IEO 2012 34 SUSPICION OF MALIGNANCY Ulceration Necrosis Bleeding Exophytic lesion Hyperkeratosis With or without white, gray, red, or brown discoloration
  35. 35. © IEO 2012 35
  36. 36. © IEO 2012 36
  37. 37. © IEO 2012 37 ABNORMAL COLPOSCOPIC Other magnification FINDINGS • Acetowhite epithelium • Punctation • Atypical vessels • Surface irregularities Colposcopy of the vulva after the application of acetic acid is discouraged as a routine examination
  38. 38. © IEO 2012 Normal findings in vulvar examination and vulvoscopy van Beurden M: BJOG 104:320;1997 40 Healthy women without vulvar symtomps Median age 37.8 (range 21 -56) In all women the vestibule resulted positive after application of acetic acid to 5%
  39. 39. © IEO 2012 39 ABNORMAL COLPOSCOPIC Other magnification FINDINGS 33% Micropapillomatosis
  40. 40. © IEO 2012 40 ABNORMAL COLPOSCOPIC Other magnification FINDINGS It can be useful Evaluation of suspicious lesions - VIN - Vulvar cancer in early stage - Warts Better delineate the margins of the lesion Identify the best site for biopsy
  41. 41. © IEO 2012 41 ABNORMAL COLPOSCOPIC Other magnification FINDINGS
  42. 42. © IEO 2012 Vulvar diseases
  43. 43. © IEO 2012 Lichen sclerosus Common inflammatory dermatoses that affect the vulva • F:M = 10 : 1 Wallace (1971): 1/300 - 1/10001 • Two peaks of presentation in women prepuberal girls and post menopausal women Aetiology: Unknown Multifactorial - genetic - autoimmune - environmental factors NOTE: Often associated with autoimmune conditions, e.g. thyroid disease, vitiligo, etc. Familial cases have been reported
  44. 44. © IEO 2012 Histology Epidermis • Epithelial/mucosal thinning • Loss of epithelial digitations Dermis • Ialinic degeneration • Oedema and inflammatory linfoplasmacellular strip between normal intermediate and deep dermis
  45. 45. © IEO 2012 Clinical Features PHYSICAL EXAMINATION •Ivory white papules or confluent plaques •Cellophane-like sheen to surface •Patchy or generalized - anywhere • vulva, perineum, perianal •Disease is not in the vagina Secondary changes - scratches, purpura, erosion - crusting, thickening (lichenification) - scarring with loss of normal architecture; fusion of labia minora -phimosis -introital stenosis
  46. 46. © IEO 2012
  47. 47. © IEO 2012 Symptoms LS has a tremendous impact on the quality of life by interfering with function and self image Most common - pruritus - can be severe, intolerable Can have soreness and burning Often asymptomatic Scratching results in open areas causing dysuria, pain, dyspareunia, etc.
  48. 48. © IEO 2012 • LS associated with vulvar squamous cell carcinoma • 5% risk – possibly overestimated • LS increases the 246-300x the relative risk of SCC • Extra genital lesions not associate with malignancy • 60% of vulvar SCC develop in a background of LS • Incidence of vulvar cancer rising in the last years • Weaker association with: • Melanoma (Friedman RJ et al, 1984) • Basal cell carcinoma (Meyrick Thomas RH et al, 1985) • Verrucous carcinoma (Brisgotti M et al, 1989) LS and malignancy Renaud-Vilmer C, Cavelier-Balloy, B, Porcher R. Vulvar Lichen Sclerosus: Effect of Long-term Topical Application of a Potent Steroid on the Course of the Disease. Arch Dermatol, Vol 140, June 2004 MacLean AB; Jones RW, Scurry J, Neill S. Vulvar Cancer and the Need for Awareness of Precursor Lesions. J Low Genit Tract Dis. 2009 Apr;13(2):115-7
  49. 49. © IEO 2012 LS and malignancy Differentiated VIN Usual type VIN VULVAR CANCER HPV Lichen sclerosus Walkden V, Chia Y, Wojnarowska F. The association of squamous cell carcinoma and lichen sclerosus; implications for follow up. J Obstet Gynecol 1997; 17: 551–3 Vilmer C, Cavelier-Balloy B, Nogues C et al. Analysis of alterations adjacent to invasive vulvar cancer and their relationship with the associated carcinoma: a study of 67 cases. Eur J Gynecol 60% 40%
  50. 50. © IEO 2012 SCC occurs in 3-6 % It is not a pre-malignant lesion but increases risk of carcinoma Chronically scarring, inflammatory dermatosis, may act as and initiator or promoter of cancerogenesis
  51. 51. © IEO 2012 Vulvar pigmented lesions Naevi Melanosis Angiokeratoma Melanoma
  52. 52. © IEO 2012 Naevi
  53. 53. © IEO 2012 Naevi
  54. 54. © IEO 2012 Cortesia, Sezione Dermatologia Università di Ferrara
  55. 55. © IEO 2012
  56. 56. © IEO 2012 Physiologic and post inflammatory hyperpigmentation
  57. 57. © IEO 2012 Melanosis Definition: benign pigmented lesion of the mucous membranes (oral, genital) characterized by: - Hyperpigmentation basal keratinocytes - Melanocytes normal or only mildly elevated - Presence of melanophages in the dermis
  58. 58. © IEO 2012 Typical features - symmetry - smooth edges - light brown - size <6 mm - often multiple Melanosis
  59. 59. © IEO 2012 In doubtful cases it may be useful dermoscopy (microscopy epiluminescence) Melanosis
  60. 60. © IEO 2012
  61. 61. © IEO 2012 Cortesia, Sezione Dermatologia Università di Ferrara
  62. 62. © IEO 2012
  63. 63. © IEO 2012 Cortesia, Sezione Dermatologia Università di Ferrara Melanoma
  64. 64. © IEO 2012
  65. 65. © IEO 2012
  66. 66. © IEO 2012 Vulvar pigmented lesions • Diagnosis can be diffucult • Dermoscopy is feasible and useful as diagnostic tool • Biopsy is mandatory in case of doubts
  67. 67. © IEO 2012 To increase the knowledge of vulvar pathology To Improve the diagnosis and treatment To Standardize terminology TAKE HOME MESSAGE ISSVD e IFCPC
  68. 68. © IEO 2012 Vaginal Intraepithelial Neoplasia (VAIN)
  69. 69. © IEO 2012 VAGINAL CANCER  rare entity (1-2 % genital neoplasia)  natural history not well known  evolutive potential of VAIN not well understood
  70. 70. © IEO 2012 VAIN natural history  23 cases  3 yrs minim. follow up  progression 9%  persistence 13%  regression 78% Aho et al., 1991
  71. 71. © IEO 2012 VaIN HETEROGENEITY RELATED TO  localizzation  VaIN grade  association with other lower genital tract neoplasia
  72. 72. © IEO 2012 VaIN localization  third superior (cervical neoplasia)  third inferior (vulvar neoplasia)  multifocal (HPV infection)
  73. 73. © IEO 2012 VaIN grade  low grade  high grade
  74. 74. © IEO 2012 Low grade VaIN  usually HPV related  focal lesion (flat condyloma)  multifocal  CIN associated  associated with vulvar warts  associated with vaginitis
  75. 75. © IEO 2012 TRETAMENT OF low grade VaIN: General principles  uncertain or low neoplastic potential  therapy in relation to the underlying disease  focal lesions can be approached by laser vaporization
  76. 76. © IEO 2012 TREATMENTO OF LOW GRADE VaIN Multifocal and diffuse  treatment of the associated condition  colposcopic follow up  laservaporization  chemotherapy (topical 5-FU)?
  77. 77. © IEO 2012 TREATMENT OF HIGH GRADE VaIN heterogeneity  primary high grade VaIN  associated with cervical neoplasia  after hysterectomy  associated to vulvar neoplasia
  78. 78. © IEO 2012 TREATMENT OF HIGH GRADE VaIN Primary VaIN  laservaporization  surgical excision (cold knife or laser)  local chemotherapy (5-FU topical)?  radiotheraphy
  79. 79. © IEO 2012 CONCLUSIONS  intraepithelial vaginal neoplasias rare  VAIN heterogeneous entity  ?therapy? Of low grade VaIN  high grade VaIN can be approached by laser or cold knife surgery  occult invasive vaginal cancer is frequently found in surgical specimens of colpectomy for high grade VaIN after hysterectomy for CIN3
  80. 80. © IEO 2012 456401
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  83. 83. © IEO 2012 456405
  84. 84. © IEO 2012 0188781
  85. 85. © IEO 2012 262821
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