Disclosures/Conflicts of Interest

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