Vulval Conditions & Skin Lesions
Amanda Oakley Dermatologist, Health Waikato Honorary Associate Professor, Waikato Clinical School President, Australian and New Zealand Vulvovaginal Society Website Manager, NZ DermNet
NZSHS 2014 Hamilton, 13 September 2014 Outline of this presentation Skin lesions Dermatoscopy Dermatological symptoms and signs Inflammatory skin diseases Diagnosis and management Vulvology Careful history and examination Make the diagnosis Take clinical images if relevant Always take written consent File securely Explain management Refer to a dermatologist &/or biopsy to confirm Follow up Photography: consent Storage of photographs Benign + malignant lesions of the vulva Benign melanocytic naevus Mucosal melanosis / lentigo Melanoma Vulval intraepithelial neoplasia Invasive squamous cell carcinoma Extramammary Paget disease What is dermatoscopy?
Hand-held device with magnification + light
Fluid immersion
Polarised Digital dermatoscopy Global dermoscopy patterns
Structureless Reticular
Globular Cobblestone Benign melanocytic naevi Proliferation of melanocytes within skin In the vulva, congenital or developmental
Well demarcated, round or oval 1-2 colours Uniform structure Structureless, reticular, globular, cobblestone patterns on dermatoscopy
Naevi have symmetrical patterns One to three patterns with symmetrical distribution Colours: white, yellow, orange, brown, dark brown, black, grey, blue, red, purple Structureless pattern
Macroscopic view Dermatoscopic view Globular pattern
Macro Dermatoscopy Cobblestone pattern
Macroscopic view Dermatoscopic view Atypical melanocytic naevi Poorly demarcated, any shape 2-3 colours Variable structure Complex / 2 patterns on dermatoscopy
Atypical naevus: complex pattern
Macroscopic view Dermatoscopic view Multicomponent naevus mons pubic Lesion monitored
2008 2010
Lines
Dots
Structureless Melanosis / melanotic macule / lentigo Skin or mucosal pigmentation (not melanocytic proliferation) Post-inflammatory, hormonal or unknown cause Pale to dark brown macules: irregular shape Multifocal and often bilateral
Dermatoscopy patterns: structureless, reticular, globular, cobblestone Focal: Parallel or curvilinear pattern Ring-like pattern Grey dots (pigment incontinence) Vulval melanosis Dermatoscopy
Circles Curved lines 85 year-old – VLS + melanosis
Structureless Vulval cutaneous melanoma 61 year-old woman No history available Large pigmented lesion in pubic skin Clinically typical of superficial spreading melanoma Dermoscopy showed chaos + clues Multicomponent: lines + clods + structureless zones Multiple colours Polymorphous vessels Referred for excision Melanoma: dermatoscopy
Multicomponent: Lines Clods Structureless zones Polarised Unpolarised
Many colours Asymmetry of structure and colour Vulval cutaneous melanoma
Macroscopic view Macroscopic view Melanoma: dermatoscopy Scar-like depigmentation Pigment network Parallel ridge pattern Melanoma Pink blush, irregular pigmentation Blue-whitish veil Vulval mucosal melanoma 66 year old woman Patient noted bleeding Extensive bilateral irregular pigmentation Ulcerated nodule Some hypomelanotic areas 7 mm thick Patient died 5 years later Melanoma + melanosis
Polymorphous vessels Structureless pattern
Vulval intraepithelial neoplasia Slowly enlarging irregular white, red, pigmented plaques Moderate, mild or no symptoms (itch, soreness) Sometimes, known history of: A) genital warts / HPV / CIN / AIN VIN (usual type) - warty, basaloid or mixed B) lichen sclerosus or lichen planus VIN (differentiated type) – associated with aggressive SCC VIN Longstanding increasing pruritus / soreness Bilateral irregular and asymmetrical erythematous / white plaque Localised hyperkeratosis + erosion Biopsy = VIN (usual-type) Lesion excised VIN Patient noticed a lump Irregular partly pigmented hyperkeratotic plaque Biopsy = VIN (usual-type) Lesion excised VIN + VLS Longstanding mild vulval lichen sclerosus Routine annual follow-up Asymptomatic asymmetrical erythematous plaque in perineum Changed on comparing with previous photographs Biopsy = VIN (high-grade, differentiated) Lesion excised VIN + VLS Longstanding vulval lichen sclerosus Itch well controlled with clobetasol propionate ointment Last year’s biopsy = LS Increasing hyperkeratosis + erosion Biopsy = VIN (high-grade, differentiated) Vulvectomy VIN + EVLP Vulval soreness poorly controlled with clobetasol Irregular hyperkeratosis + erosions Bx VIN (usual type) Previous bx EVLP VIN (low-grade): dermatoscopy VIN: pigmented Longstanding severe VLS Routine follow-up Heavy smoker Irregular pigmented plaque Dermatoscopy: chaos + clues ?melanoma Previous VIN x 2 (differentiated) Excision bx Dx VIN (usual-type)
VIN: dermatoscopy Asymmetry of colour + structure Multicomponent Lines, dots, structureless zones Light brown, dark brown, black, grey, red
Invasive squamous cell carcinoma Arises within VIN or within a chronic inflammatory skin disease, especially lichen sclerosus (6%) Enlarging irregular, hard nodule, often ulcerated May be multifocal Often aggressive Invasive squamous cell carcinoma
SCC + VIN + VLS Bilateral SCC + VLS Invasive squamous cell carcinoma
HPV-associated HPV-associated + VIN Invasive squamous cell carcinoma Longstanding VLS Bleeding ‘piles’ o/e anal carcinomas (2-3) HPV-associated 2 previous primary vulval SCC (vulvectomy, lymphadenectomy + radiation) Previous melanoma Multiple intraepidermal carcinomas on limbs Tx radiotherapy Extramammary Paget disease Intraepidermal or epithelial adenocarcinoma Underlying / associated invasive cancer Slowly growing discrete plaque Often multifocal Border may appear defined yet recurrence is usual after wide excision Paget disease Longstanding pruritus Large irregular unilateral purplish plaque with superficial erosions Surgically excised Paget disease: recurrence Multiple recurrences treated surgically Trial of imiquimod for 16 weeks Complete remission Small recurrence, successfully retreated Paget disease: recurrence Extensive bilateral painful and itchy eroded plaques Multiple previous surgeries Further operations considered but unlikely to be curative Treated with repeated 16- week courses of imiquimod cream Severe adverse reactions Partial response to imiquimod
Symptoms markedly improved Melanoma Rare in vulva Affects elderly women Most are cutaneous melanomas Mucosal melanoma similar to acral melanoma Not related to skin colour or sun exposure Mild or no symptoms Best diagnosed using dermatoscopy Vulval mucosal melanoma 81-year old woman Mild pruritus Amelanotic unilateral plaque No dermatoscopy performed 3-mm thick melanoma Excised + lymphadenectomy Patient remained well Neoplastic lesions of the vulva If in doubt, multiple and generous biopsies Surgical management for suspected malignancy
But many lesions are benign … Dermatoscopy helps reduce unnecessary surgery Seborrhoeic keratosis Found on hair-bearing skin Irregular structure despite benign nature In contrast to viral warts Curved thick brown lines –maze-like pattern ‘Cerebriform’ In contrast to dermal naevi – cobblestone pattern White and orange clods ‘Milia-like cysts’ & ‘comedo-like openings’ Also seen in dermal naevi Seborrhoeic keratosis White / orange clods Stuck-on appearance Red dots in curved lines
Macro view Dermatoscopy view Genital warts: numerous lobules
Red dots surrounded by white circles within white or pink clods Molluscum contagiosum
Umbilicated papules Shiny white clods
Macro view Dermatoscopy view Angiokeratomas Angiokeratomas Haemorrhage in lichen sclerosus Vulval structures – Fordyce spots An unusual lesion—no dermatoscopy Excised Hidradenoma papilliferum An (extra)mammary cell proliferation (benign) A common lesion—Bartholin cyst Swelling noted Recent discomfort Treated surgically Dermatological symptoms Pruritus (itch) Soreness / stinging / burning / pain Dyspareunia / apareunia Asymptomatic signs Evaluation of signs How old is the patient? What site is affected? Any anatomic abnormality? Colour: red, white Primary lesions Macules, papules, plaques, vesicles Secondary lesions Fissures, excoriations, haemorrhage, crusting Causes of vulval itch Candida albicans infection Irritant contact eczema Seborrhoeic dermatitis Psoriasis Lichen simplex Lichen sclerosus Lichen planus
Pruritus Maybe unremitting and extremely severe Scratching may cause lichenification, whatever the cause Assess using linear analogue scale
0 10 No itch Severe itch Location may help diagnosis Lichen simplex May be unilateral Dominant side Lichen simplex Chronic intensely itchy lichenified dermatitis Consider potential causes of chronic itch Irritant dermatitis, psoriasis, neuropathy Treat with potent topical steroid ointment Betamethasone valerate, methylprednisolone, mometasone Daily for 4 to 6 weeks then weekend pulses May require ultrapotent topical steroid Clobetasol, betamethasone diproprionate May require tricyclic if neuropathic origin likely
Lichen simplex Perineum - ?Candida Skin thickening Fissuring Skin coloured Dyspigmentation common
Lichen simplex Unilateral, labium major Due to rubbing intense itch Well-demarcated Skin thickening Increased skin markings Skin coloured Dyspigmentation common
Lichen simplex May be bilateral Thickened skin Prominent skin markings Broken off hairs
Lichen simplex Bilateral – ?psoriasis Skin thickening Increased skin markings Skin coloured Dyspigmentation common
Location may help diagnosis Candida albicans infection Candida albicans & dermatology Candida albicans associated with: Infection – vaginal or vulvovaginal or intertrigo Dermatitis – metabolites are irritating to skin, activating innate immune system Psoriasis – macerated skin is colonised, acts as superantigen Lichen planus – anticandidal therapy can be of benefit Pruritus: Candida albicans infection Mostly affects young women & diabetics May complicate other skin disorders Yeasts found in vagina, occasionally mucosal vulva Symptoms in & around vagina Often flares premenstrually Candida albicans – vaginal Candida albicans – vulvovaginal Candida albicans infection Irritant contact dermatitis May spread to labia majora Scratching may lead to lichen simplex Candida albicans – irritant dermatitis Candida albicans - lichen simplex Candida albicans: treatment Confirm with high vaginal swab Topical therapy vs. oral azole Topical: inexpensive but irritant Oral: convenient but expensive, drug interactions Hydrocortisone to treat the dermatitis Location may help diagnosis Irritant contact eczema Eczema is usually “irritant” Cutaneous not mucosal: labia majora Affects all ages including prepubertal girls Irritant contact dermatitis from soap, over-washing, urine Allergic contact dermatitis is much less common Mostly improves with mild topical steroid short term Explain how washing / soap / scratching may aggravate Supply emollient / barrier cream Irritant contact dermatitis Burn-like Glazed erythema Superficial erosions Oedema
Faecal and urinary incontinence Irritant contact dermatitis Erythema Oedema Fissuring
Soap, water, rubbing Irritant contact dermatitis: treatment Reduce contact with irritants Napkins, hygiene, nursing care Barrier creams, soap substitutes Avoid unnecessary washing, wiping Hydrocortisone cream / ointment Stronger topical steroids are not particularly effective Soothing emollients frequently
Location may help diagnosis Seborrhoeic dermatitis Seborrhoeic dermatitis Vulval seborrhoeic dermatitis often seen in association with seborrhoeic dermatitis affecting other sites Poorly-demarcated salmon-pink thin plaques Mons pubis and flexures May or may not be scaly Mild or absent symptoms Seborrhoeic dermatitis Seborrhoeic dermatitis
Axilla Seborrhoeic dermatitis Location may help diagnosis Psoriasis Psoriasis Vulval psoriasis often seen in association with psoriasis affecting other sites, especially scalp and flexures Well-demarcated erythematous plaques External aspects of vulva and flexures May or may not be scaly Absent to severe symptoms Vulval psoriasis Vulval psoriasis ?+irritant dermatitis Vulval psoriasis ?+ candida Psoriasis + lichen sclerosus Psoriasis less symptomatic but more impressive Psoriasis ‘outside’ Lichen sclerosus ‘inside’ Psoriasis does not alter anatomic features Lichen sclerosus can be destructive Psoriasis
Circumscribed erythematous scaly plaques Flexural psoriasis Location may help diagnosis Lichen sclerosus Pruritus: lichen sclerosus Mainly labia minora, clitoral hood, perineum Perianal in 50% Extragenital in 15% Most are 50 years or older but children may be affected May have associated autoimmune conditions 25% thyroid antibodies; 20% hypothyroid 10% psoriasis 5% morphoea, vitiligo, lichen planus Lichen sclerosus Itch: nil to very severe Soreness: nil to very severe Dyspareunia / apareunia Itch Soreness Fissuring due to sclerosis Introital narrowing due to agglutination / bridging Lichen sclerosus Distribution: figure of 8 with wings Usually bilateral Sometimes unilateral Colour – white,crinkly (‘cigarette-paper’) Sclerosis - firm Anatomic change - destructive
Lichen sclerosus Labia minora / perineum Spotty white
Lichen sclerosus Labia minora / perineum /perianal / labial sulci White spots
Lichen sclerosus White, like vitiligo Anatomic change Loss of labia minora
Lichen sclerosus LS + vitiligo Anatomic change Loss of labia minora
Lichen sclerosus Perianal / perineum White spots Lichen sclerosus Distorted clitoral hood / labia minora Postinflammatory white areas Postinflammatory brown pigmentation Lichen sclerosus Resorption of labia minora Fissures in labial sulci, perineum Lichen sclerosus May involve crural creases White plaques Fissures in sulci Lichen sclerosus Flattening of clitoral hood Resorption of labia minora Haemorrhages Lichen sclerosus Flattening of clitoral hood Resorption of labia minora Lichen sclerosus Distortion of labia minora Ulceration Haemorrhage Lichen sclerosus Bridging / fusion / agglutination of left and right labia Introital narrowing Lichen sclerosus Bridging / fusion / agglutination of left and right labia Ulceration Lichen sclerosus Severe introital narrowing Splitting of posterior forchette Lichen sclerosus Haemorrhage in posterior forchette Lichen sclerosus Haemorrhage right labia minora Lichen sclerosus Ulcer right labium minor Lichen sclerosus Hyperkeratosis Consider biopsy DD VIN Lichen sclerosus Hyperkeratosis Consider biopsy DD VIN Lichen sclerosus: extragenital Lichen sclerosus: treatment Ultrapotent topical steroid, usually clobetasol ointment Once daily until clear (one to three months) Then alternate days for the same duration Then once or twice per week long-term or PRN Adjust frequency if necessary Explain to patient precisely where to apply the cream Clinical photographs are useful for follow-up Lichen sclerosus – follow-up Review after 3 months Then after 6 months Then annually if symptomatic or requiring treatment To ensure adequately treated To ensure not over-treated To assess for VIN, LP and invasive SCC If stable and no complications, follow-up can be in primary care Lichen sclerosus: other treatment Treatments that sometimes work Milder topical steroids Calcineurin inhibitors (expensive; may burn) Hydroxychloroquine 200mg bd: 6-month trial Acitretin 25mg daily x 3 to 6-month trial (many adverse effects) Prednisone 40mg daily for one month reducing
Treatments that don’t usually work Calcipotriol cream Methotrexate
Pruritus: lichen planus Various clinical presentations Cutaneous Mucosal Mixed Most are adults May also have lichen sclerosus Absent to very severe symptoms Treatment-resistant Lichen planus: cutaneous type Violaceous plaques Wickham’s striae Labia majora Irregular distribution Typical LP elsewhere Lichen planus: cutaneous type Violaceous plaques Postinflammatory pigmentation may be marked Irregular distribution Location may help diagnosis Erosive lichen planus Unilateral or bilateral Diffuse or patchy Plasma cell vulvitis Soreness Soreness associated with inflammatory skin disease may be mild tenderness or stinging, to very severe pain Pain may only be associated with intercourse (dyspareunia), and can prevent it (apareunia) Erosive lichen planus > lichen sclerosus
0 10 No pain Severe pain Lichen planus: mucosal type May be very itchy More often very painful Exquisitely tender Destructive – loss of clitoral hood, labia minora May cause introital narrowing More likely to involve vagina (unlike lichen sclerosus) May also affect oral mucosa Buccal mucosa, inner lips, tongue Desquamative gingivitis Oral lichen planus Lichen planusL gingivitis Erosive lichen planus Red glistening painful mucosal patch Loss of labia minora Erosive lichen planus Erosive lichen planus Mucosal disease may result in postinflammatory pigmentation Erosive lichen planus Bridging may be due to concomitant lichen sclerosus or to the lichen planus Erosive lichen planus Destruction of tissue Introital narrowing Eaten-away appearance Erosive lichen planus Mixed picture Eaten away right introitus Eroded left introitus Erosive lichen planus: treatment Topical clobetasol daily May be required daily long-term Topical calcineurin inhibitors, e.g., pimecrolimus cream Sometimes extremely effective Oral prednisone 40mg daily for 2-3 months then reduce over 2-3 months Methotrexate 15mg once weekly for 6-month trial; sometimes can be stopped if symptoms have gone into remission Often symptoms respond better than signs
Erosive lichen planus: other oral tx Hydroxychoroquine Itraconazole Griseofulvin Ciprofloxacin Metronidazole
Plasma cell vulvitis Rare histological diagnosis when you think patient has LP Mild to moderate symptoms Introital / vaginal haemorrhagic red patches Non-destructive
Plasma cell vulvitis Deeper red than LP Haemorrhagic More internal More patchy Plasma cell vulvitis May extend periurethrally Contact bleeding Plasma cell vulvitis: treatment Topical antibiotic Clindamycin cream Metronidazole cream Hydrocortisone cream Emollients for vulva Replace soap by aqueous cream or other non-irritating cream or liquid cleanser Cetomacrogol cream or sorbolene for soothing an itch Apply frequently Petroleum jelly or fatty cream suitable if incontinent Apply frequently Incontinence pads and pants are mostly excellent but sometimes irritate (beware panty liners designed for another purpose) Water-based lubricants for sexual intercourse only Adverse effects of topical steroids Atrophy Striae (stretch marks) Telangiectasia (prominent blood vessels) Periorificial dermatitis Burning discomfort Bright red skin Often: spotty margin or erythematous streaks
Steroids have usually been prescribed for minor reason, or applied to incorrect site or used more frequently than necessary Rare when used properly (once daily, thin smear, to affected area only) Steroid atrophy Periorificial dermatitis
Lichen sclerosus Periorificial dermatitis Psychosocial factors & the vulva Culture, ethnicity, religion Age, education, beliefs and fears Marital status, family support Severity of symptoms
Most women present late, and in distress, fearing the worst Results of our survey of the impact of vulval disease are disturbing (to be reported on another occasion) You are all invited to: Join the Australian and New Zealand Vulvovaginal Society Attend our scientific meeting 14-16 November 2014, Perth
www.anzvs.org