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Vulvar Ulcers ULCERS painless painful Vulvar Ulcers Lyne/e J. Margesson MD FRCPC Interna<onal Congress of Dermatology ISSVD Sister Society Mee<ng Buenos Aires, Argen<na syphilis April 14, 2017 Aphthae NO CONFLICTS Ulcer: Ulcer Erosion Result of full thickness loss of epidermis painful extending into dermis or deeper • Always secondary phenomenon • From Destruction, Necrosis, Inflammation (as in syphilis, aphthae, or tumor) 1 Erosion VULVAR ULCERS Result of loss of all or part of epidermis only ■ MULTIPLE causes spanning a variety of ages, ethnic groups, geographical regions • Result of broken vesicles, pustules or bullae ■ Differing pathologies may co-exist ■ NOT SPECIFIC ■ Classification of vulvar ulcers is difficult Vesicle or Bulla - Intraepidermal - contact dermatitis, Staph Sub epidermal - bullous disease, drug eruption Pustule: Candida, HSV Causes of Vulvar Ulcers Syndromic Approach § Used in areas where no good testing is available Infectious ulcers - commonest STIs # 1 – HSV § Lessens the dependence on laboratory tests # 2 – Syphilis (saves time and money) # 3 - Chancroid - Africa – chancroid Granuloma Inguinale - New Guinea – very rare North America § Simplifies a complex diagnostic process Non-infectious ulcers § Permits the treatment to begin immediately - Skin disorders – Aphthae, Crohn’s Disease, Trauma, cancer § STDs included are variable depending on locally prevalent STDs 2 Syndromic Protocol for Genital Ulcer Disease Commonest Causes of Vulvar Ulcers Patient complains of genital sore Infectious Non-infectious Counsel Take history and examine No Herpes simplex immunosuppressed Aphthous ulcers Educate Genital ulcer / blister present? Promote / provide condoms Contact Dermatitis Yes Syphilis – more common Small blister(s) Human Immunodeficiency Virus Crohns disease or Yes small ulcer(s) and Herpes Management Trauma history of recent blisters? Drug No Malignancy Single small ulcer and painful Yes Treat for LGV, matted gland? chancroid Squamous Cell Carcinoma • Look for other STDs No (if present use appropriate flowchart) • Aspirate any fluctuant glands • Counsel on compliance and risk Treat for syphilis and chancroid reduction Uncommon Vulvar Ulcers • Promote and provide condoms • Notify partner(s) and treat Chancroid Behcets disease – rare in North America • Take blood for RPR/VDRL Langerhans Cell Histiocytosis • Ask to return after one week Granuloma Inguinale Lymphogranuloma venereum 25-Apr-17 Herpes Simplex Virus (HSV) HSV Commonest cause of vulvar erosions / ulcers Vesicles Ulcers are seen in the Immunosuppressed Primary HSV Pustules Erosions - Reversal of HSV II/I ratio - for new cases 50 % HSV I - Usually spread from an asymptomatic partner Recurrent HSV - Women are unaware of their infection - Most cases present with recurrent HSV with no primary HSV history • • 3 • Very painful and HSV in Immunosuppressed usually indolent HSV in Immunosuppressed Herpes Simplex Virus (HSV) see Chronic ulcers extending at Diagnosis: periphery PCR, culture, biopsy, Necrotic Type-specific serology, Tsank smear From Dr Peter Lynch Think of HSV in a patient with non-healing genital, very painful punched out ulcers From Dr Peter Lynch From Dr. Libby Edwards Syphilis Syphilis – Treponema Pallidum • cases USA 2016 - 23,872 an increase of 19 % in 1 year Chancre: painless ulcer of Primary Syphilis • Starts as a papule, enlarges and ulcerates. • Incubation 9 - 90 days (average 21 days) • Sharply demarcated, raised and firm edges • Solitary or multiple • Lymphadenopathy in 80% : nontender firm nodes • Painless ulcer –chancre • Usually on cervix • Sharp edge • Seldom seen • Clean base • Duration – 3 - 6 weeks 4 Dr Claudia Marchitelli Syphilis is on the rise Syphilis - Diagnosis Find out from your lab what testing is used Treponemal tests Specific anti-treponemal antibody tests – Treponema Enzyme Immunoassays (EIAs) Non-treponemal tests – RPR and VDRL Biopsy and stain – silver stain (Warthin-Starry) 5 Syphilis - Diagnosis Tradi'onal Algorithm Reverse Sequence Algorithm Severe Irritant Treponemal Immunoassay (EIA / CIA) RPR/VDRL Contact Negative: Positive: No further RPR/VDRL Testing Dermatitis Negative: Positive: No further testing Treponemal Test Positive: No further testing Treponemal specific tes'ng: - Enzyme-immunoassays (EIAs) Negative: Confirmatory Trichloracetic - Chemiluminescence immunoassay (CIA) Treponemal Test (TPPA) acid - Treponema pallidum par<cle agglu<na<on (TP-PA) Non-Treponemal tests A caustic burn - RPR and VDRL TRAUMA Diaper Rash Urine + Feces Blunt / sharp Factitial Chemical Mechanical Physical 20% heat Factitial benzocaine 6 Quadriplegic in Pressure Ulcers wheel chair Radiation Dermatitis and Ulcer Dr A Lev-Sagie Israel Amyotrophic Lateral Sclerosis Dr Hope Haefner TUMORS Histiocytosis Dr Nina Madnani * * Squamous Cell Carcinoma (in LS) Histiocytosis Squamous Cell Carcinoma (in LP ) * Painful oral and genital ulcers. LS, HSV, SCC Vulvar Langerhan s Cell Histiocytosis Abbas O, Shbaklo Z, Kibbi AG, Rubeiz N. Clin Exp Dermatol. 2010 Jun;35(4):e184-6. 7 Secondary Aphthae Vulvar Aphthous Ulcers Acute (more common) – can recur - Usually a prodrome: fever, headache, malaise, GI upset - Very common cause of acute painful vulvar ulcers in - EBV, Mycoplasma pneumoniae, viral upper respiratory infection North America and Europe (parvovirus, influenza , paramyxovirus) or gastroenteritis (salmonella), toxoplasmosis gondii, Strep, mumps, CMV, Lyme • Primary/idiopathic (85-90%) or secondary Chronic or Recurrent/Complex (recurrent oral & genital aphthae) • Simple: Inflammatory Bowel disease: Crohns, Ulcerative colitis, Celiac disease Behcet’s disease Recurrent, minor, major or herpetiform aphthae Medications: cytotoxic, NSAIDs Distinct ulcer-free periods Myeloproliferative disease, cyclic neutropenia, lymphopenia • Complex HIV Almost constant - oral aphthae + vulvar ulcers Syndromes (rare) Recurrent oral/genital aphthae without systemic Sweet’s Syndrome PFAPA – periodic fever, aphthae, pharyngitis, adenitis symptoms MAGIC – mouth and genital ulcers with inflamed cartilage Aphthous Ulcers: Pathogenesis Vulvar Aphthous Ulcers • Cause is unknown – ■ Present most commonly as acute, Mostly reactive triggered by various antigens painful, reactive ulcers in • Infections may trigger aphthae; but do not directly younger patients cause the lesions Synonyms: Hypothesis: microbial antigens, by way of molecular § Ulcus vulvae acutum mimicry, induce a cytotoxic immune response § Lipschütz ulcers - a local vasculitis § Reactive nonsexually related acute genital ulcers § Nonsexually acquired genital ulceration ( NSAGU ) § Complex aphthosis 8 Dr.Libby Edwards Vulvar Aphthous Ulcers Can be acute, chronic +/- recurrent - more commonly acute • Sudden onset • Usually multiple, painful, well demarcated punched-out ulcers • Size: most < 1cm; can be 1 - 3 cm • Prodrome - flu-like with mild fever, headache, malaise • Duration 1 - 3 weeks - can last months • One episode, less common recurrent – 30% adults recur • Past history of oral aphthae – canker sores • Rarely Behcets in North America Major Aphthae Herpetiform aphthae Aphthous Ulcers Aphthae from Mycoplasma pneumoniae 9 Evaluation Vulvar Aphthae Thorough history and physical – eye, oral, genital MAY NOT NEED TO TEST EXCEPT FOR HSV and SYPHILIS For acute prodrome, if indicated • CBC, diff • Serology for HSV, HIV, EBV, syphilis, CMV, No Biopsy • Mycoplasma pneumoniae Usually • Influenza – swab PCR needed • HSV - swab for PCR – ALWAYS R/O HSV • For Strep -throat swab and antistreptolysin O titer For recurrent ulcers • GI investigations for inflammatory bowel disease • CBC For myeloproliferative diseases Diagnosis of exclusion – etiology seldom found Vulvar Aphthae – Therapy § Pain control – topical, systemic § Prednisone 40 – 60 mg each morning Rx HSV if (5 - 10 days, then ½ dose 5 - 10 days ) unsure - ultrapotent corticosteroid § Educate -Most often a one-time event, can recur If not controlled – recurrent : Intralesional triamcinolone 5-10 mg/ml doxycycline 50-100 mg daily colchicine 0.6 mg bid-tid if tolerated dapsone 50-150 mg per day or dapsone + colchicine pentoxyfylline 400 mg tid cyclosporine 100 mg 1-3/d thalidomide 100-150 mg per day TNFα inhibitors (adalimumab, infliximab) 10 Epstein-Barr Virus in 15 year old EBV in 17 month old Clinical- acute onset - fever, malaise, sore throat – baby - recent hx of mono in family member Lipschütz ulcer in a 17-month-old girl: a rare manifestation of Epstein-Barr primoinfection. Burguete Archel E, Ruiz Goikoetxea M, Recari Elizalde - Diagnosis: E Eur J Pediatr. 2013 Aug;172(8):1121-3. Serology for IgM antiviral capsid antigen antibodies (VCA-Ab) Vulvar Crohns Disease Chronic inflammatory bowel disease that can have Knife Cut and aphthous vulvar ulcers Patterns: Non-contiguous/metastatic - 90% vulvar lesions -Classic knife cut ulcers - Labial edema +/- ulcers - Perianal tags Contiguous – direct fistulae from bowel to skin Non-specific Crohn s - aphthae – oral and vulvar - hidradenitis suppurativa type lesions and pyoderma gangrenosum Knife Cut Ulcers 11 Vulvar Crohn’s Disease (CD) 16 yrs vulvar swelling pain ulcer • Rare: 132 vulvar CD cases reported since 1965 • Average age of onset 28 - 34 yrs • Vulvar CD can precede GI Crohn’s in 25% • 20 % women with CD have vulvovaginal complaints • Vulvar CD without GI disease often missed Crohn’s Disease Vulvar Crohn’s Disease • Symptoms – often asymptomatic - 35 – 75 % vulvar soreness / pain - 40 % itch - 60% swelling - Other: vaginal discharge, dysuria Signs – - Edema / swelling, lymphangiectasia (anal tags) - Ulcers -
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