Your Diagnosis Is? Test Your Knowledge of Various Vulvovaginal Conditions Chicago October , 2013 Hope K. Haefner, MD Disclosures/Conflicts of Interest Hope K. Haefner, MD Advisory Board of Merck, Co. Inc. Off label us of multiple medications discussed 1 Written Information Available: University of Michigan Center for Vulvar Diseases (Google) http://obgyn.med.umich.edu/patient-care/ womens-health-library/vulvar-diseases Then, click on Information on Vulvar Diseases 2 Course Objectives At the end of this course, the participant should be able to: • Identify the clinical features of various vulvovaginal conditions • Become familiar with a variety of treatments for skin diseases 3 Gross and histologic images 4 Test Format The image shown represents which vulvar condition? Test Format The image shown represents which vulvar condition? A  B  C  D  Vulvar intraepithelial neoplasia Melanoma Molluscum contagiosum None of the above d 5 A 20 year old G0 is referred to you with papillary projections on her vestibule 6 7 Your diagnosis is? (Part 1) A  B  C  D  Fordyce spots Micropapillomatosis Condyloma Inclusion cysts Your treatment is? (Part 2) A  B  C  D  No treatment needed Laser Sharp excision Trichloroacetic acid 8 A 64 y.o. G4P4 was recently diagnosed with lichen sclerosus (no biopsy performed). She was started on clobetasol propionate. She calls complaining of vulvar pain. 9 Your diagnosis is? A. Lichen planus B. Pemphigoid C. Lichen sclerosus with herpes infection D. Invasive squamous cell carcinoma 10 How many different types of herpes exist that affect humans with disease? A. B. C. D. 2 4 6 8 http://en.wikipedia.org/wiki/Herpesviridae 11 What percent of people with HSV-2 are unaware that they are infected? A. B. C. D. 10-20% 21 – 40% 50- 70% Over 80% 61-year-old G3P3 presents with constant vulvar drainage * 12 If you could only look at one other area of her body, where would you look? A. B. C. D. Eyes Colon Axilla Mouth 13 What is this? A. B. C. D. Squamous cell carcinoma Epithelial inclusion cyst Pyogenic granuloma Paget’s disease 14 What is the staging system used for Hidradenitis Suppurativa? A. B. C. D. WHHS (World Health Hidradenitis Staging) Clark’s Staging Breslow’s Staging Hurley’s Staging Hurley’s Criteria for HS Staging Stage I: abscess formation, single or multiple, without sinus tracts and cicatrization. Stage II: recurrent abscesses with tract formation and cicatrization. Single or multiple, widely separated lesions. Stage III: diffuse or near- diffuse involvement, or multiple interconnected tracts and abscesses across entire area. 15 HS Clinical Features  Chronic lesions “Tombstone comedones” – open twin or multi-headed comedones Chronic draining, malodorous sinuses Dense fibrous scars Pitted and pocked acne-like scars 16 17 18 19 4 months post op from skin grafts 20 2 years after surgery A 79 y.o. G1P1 is referred for vulvar lesions These have been present for years. Rare itching—none currently 21 Your diagnosis is? A. Epithelial inclusion cysts B. Invasive squamous cell carcinoma C. Fordyce spots D. Hidradenitis suppurativa 22 She is not bothered by them; Your treatment is? A. B. C. D. Wide local excision Radical vulvectomy No treatment needed Baby shampoo to cysts 23 Another condition associated with a “cheesy substance” is? A. Necrotizing fasciitis B. Bartholin duct cyst C. Vulvar intraepithelial neoplasia D. Molluscum contagiosum * 27 y.o. with 1 1/2 year history of vulvar irritation. History of genital herpes. PMH significant for hypothyroidism. 24 She had tried multiple agents for her condition including topical steroids, Vagisil, antibiotics, and Diflucan. She used oral steroids but developed knee pain. Protopic has not helped. 25 26 27 MICROSCOPIC DIAGNOSIS: 1. Vulva, biopsy: Hyperkeratosis consistent with lichen simplex chronicus. 2. Right labium majus, biopsy: Scar with overlying and adjacent lichen simplex chronicus. 3. Vulva, left bottom, biopsy: Excoriation with lichen simplex chronicus. 28 Skin biopsy: Site: Vulva; Lesional status: Lesional: No evidence for pemphigus/pemphigoid. Occasional cytoid bodies suggestive of lichen planus. Your diagnosis is? A  B  C  D  Lichen planus Pemphigoid Molluscum Severe contact dermatitis 29 Which agent has she reacted to? A  B  C  D  Diflucan Topical steroids Vagisil Antibiotics 30 31 VULVAR FISSURES • Two main varieties • Posterior fourchette fissures which occur with intercourse • Skin fold fissures which patients describe as “paper cuts” 32 Treatment • Rule out candida, lichen sclerosus, atrophy, herpes, Crohn disease • Reduce friction – Ample lubricants (water, silicone, oil based) – Position changes • Treat atrophic vaginitis with local estrogen and dilator • Treat vestibulodynia and vaginismus • Surgical excision as last resort 33 Treat with Dilators Splash White Bottle Drying Rack by Skip Hop More Thoughts on Drying Racks Munchkin Sprout Drying Rack Handmade! Target 34 Treat with Dilators… If cost is an issue Surgical Therapy for Fissures • Close anterior to posterior 35 A 56 y.o. G3P3 presents with vulvar irritation. 36 D. Birenbaum MD collection What is your diagnosis? A  B  C  D  Candidiasis Bacterial vaginosis Trichomoniasis Desquamative inflammatory vaginitis 37 Atrophic Vaginitis Desquamative Inflammatory Vaginitis (DIV) Etiology is unknown; theories • Lichen planus • Nonspecific term for any erosive mucosal disease (LP, pemphigus vulgaris, cicatricial pemphigoid) • Group B streptococcal infection • Specific sterile inflammatory vaginitis, probably autoimmune • Common picture of several uncharacterized diseases 38 DIV Therapy - Intravaginal clindamycin cream - If that fails, clindamycin cream and hydrocortisone suppositories - If no response, compound a high dose intravaginal corticosteroid and 2% clindamycin 15-year-old girl who had sudden onset of dysuria and severe vulvar burning. -She was feeling tired. -She has a cough, a low-grade fever and malaise. -Her doctor diagnosed acute HSV and started her on acyclovir and sent her to be seen. This is day 3. 39 Your Diagnosis Is? A. B. C. D. Aphthous Ulcers Atypical Herpes Simplex Virus Drug Rash Trauma – Abuse? 40 Aphthous Ulcers Vulvar Aphthous Ulcers Canker sores on the vulva Acute painful ulcer(s) of sudden onset Common as acute reactive ulcers in younger patients - often missed Synonyms:  Ulcus vulvae acutum  Lipschütz ulcers  Reactive nonsexually related acute genital ulcers* * Lehman JS et al. J Am Acad Dermatol; 2010;63:44-51 41 Aphthous Ulcers: Pathogenesis     Cause is unknown - Idiopathic -90% Secondary 10% Genetic factors: positive family history in some cases Infections may trigger aphthae; but most likely do not directly cause the lesions Hypothesis: microbial antigens, by way of molecular mimicry, induce an autoreactive (autoimmune) process Vulvar Aphthous Ulcers “Canker Sores” on the Vulva • • • • • • • • • Average age is 14 (9-19) yrs 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 Past history of oral aphthae – canker sores Rarely Behcet’s in North America Huppert JS Lipschutz ulcers: evaluation and management of acute genital ulcers in women. Dermatol Ther. 2010 Sep-Oct;23(5):533-40 Lai K, Lambert E, Mercurio MG. Aphthous vulvar ulcers in adolescent girls: case report and review of the literature. J Cutan Med Surg. 2010 Jan-Feb;14(1):33-7. Review. 42 Aphthae from Mycoplasma pneumoniae APHTHAE Acute (more common) – usually a prodrome - fever, headache, malaise, GI upset - EBV, Mycoplasma pneumoniae, viral upper respiratory infection or gastroenteritis, influenza, Strep, CMV Recurrent /Complex (recurrent oral and genital aphthae) Inflammatory Bowel disease - Crohn’s, Ulcerative colitis, Celiac disease Behcet’s disease Medications – cytotoxic, NSAIDs Myeloproliferative disease, cyclic neutropenia, lymphopenia HIV Syndromes – rare PFAPA – periodic fever, aphthae, pharyngitis, adenitis MAGIC – mouth and genital ulcers with inflamed cartilage 43 Aphthous Ulcers 44 Evaluation Vulvar Aphthae Thorough history and physical – eye, oral, genital Lab tests – CBC, diff Serology for HSV, HIV, EBV, syphilis, CMV, Mycoplasma pneumoniae Influenza – swab PCR HSV - swab for PCR For Strep -throat swab and antistreptolysin O titre Tests as indicated for – RARE- paratyphoid and typhoid (stool, blood culture), TB enterocolitis, Yersinia GI investigations – for inflammatory bowel disease and celiac disease Diagnosis of exclusion – etiology often not found Vulvar Aphthae – Therapy Pain control – topical, systemic Prednisone 40 – 60 mg each morning until pain resolves (3-5 days, then ½ dose 3-5 days ) - ultrapotent corticosteroid Educate -Most often a one-time event, can recur If not controlled: Intralesional triamcinolone (Kenalog 10) 5-10 mg/ml colchicine 0.6 mg bid-tid if tolerated dapsone 50-150 mg per day dapsone + colchicine pentoxyfylline 400 mg tid cyclosporine 100 mg 1-3/d thalidomide 100-150 mg per day 45 Summary When patients do not respond to therapy –Reconsider the diagnosis –Check for infection - fungal, bacterial, HSV –Consider contact dermatitis to a medication, over washing, etc. –Evaluate for carcinoma 46 Great Job! Questions and Answers 47