Ulcers of the vulva



Download 37.49 Kb.
Date conversion31.01.2017
Size37.49 Kb.
Ulcers

Hope K. Haefner, M.D.

Professor of Obstetrics and Gynecology

Co-Director, The University of Michigan Center for Vulvar Diseases

The University of Michigan Hospitals
ULCERS OF THE VULVA
Ulcers of the vulvar are diagnostically challenging. It is often very difficult to differentiate them from erosions. Erosions involve loss of the epidermis only, not the dermis, and they appear as deep red, often weeping, patches. Ulcers are deeper, extending into the dermis with a white or yellowish fibrinous base. Most diseases produce either erosions or ulcerations but often these overlap. Erosions can be transformed into ulcers by secondary infection, irritant contact dermatitis, rubbing and other trauma.
The best example is severe herpes simplex virus (HSV) infection. The primary lesion of HSV is an intraepidermal vesicle that becomes a pustule that ruptures, creating an erosion. When severe, these erosions can ulcerate. An ulcer is characterized by loss of both epidermis and dermis.
A diagnosis of a vulvar ulcer based on morphology alone is erroneous 40% of the time. Laboratory testing is usually required.

DIFFERENTIAL DIAGNOSIS

In sorting out these conditions, try to identify the primary process. Is it a pustule within the epidermis as in candidiasis or herpes simplex, an intraepidermal vesicle in acute eczema (contact dermatitis), or a frank bulla (intraepidermal or sub-epidermal) as in the bullous diseases or drug eruptions. All these rupture, resulting in erosions and/or ulcerative disease.


All of these can look much alike and it can be very difficult to differentiate them clinically, especially if there are secondary changes with crusting and bleeding, etc.
A good history is important, as is the understanding that the history may be inaccurate. Many women have problems with discussing the genital area

Note the following factors:

Age Immune status

Epidemiology and demographics of their community Systemic disease

Travel and sexual exposure History of abuse

Pattern of recurrence Previous sexually

Previous/present treatment transmitted diseases

Note the following factors specific for vulvar ulcers:

Pain Systemic symptoms

Induration Lymphadenopathy

Friability Fever

Number of lesions (single or multiple) Malaise

Acute or chronic Headache

Speed of onset Extragenital changes


Tests for all ulcers: - HSV culture - CBC

- Candida cultures - RPR (syphilis screen)

- HIV screen

Serology for Epstein Barr virus (EBV) - antiviral capsid antigen – IgM for EBV and Serology for Mycoplasma Pneumoniae

- Biopsy for H&E +/- immunofluorescence
Consider more extensive workup depending on the case, e.g. cultures, smears and serology.
Biopsies are very important. Always biopsy the edge of the lesion – not the necrotic center. A wedge excision of the edge often gives the best information for the pathologist but may be impractical. Two smaller punch biopsies may be more appropriate

Why biopsy? Because it is impossible to guess the cause of most ulcerative erosive conditions – biopsy gives the most information, especially for chronic ulcers. Although it is an uncomfortable procedure it can be made almost painless. One is adding an extra open area to an already tender area but your patient is already very stressed and wants to know the answer.


Most common causes of primary vulvar ulcers ( not erosions):

INFECTIOUS

Venereal

Non Venereal

Herpes simplex (HSV)

EBV

Immunosuppressed

Mycoplasma pneumoniae

Chancroid




Granuloma inguinale




Lymphogranuloma venereum




Syphilis




Human immunodeficiency virus




NON-INFECTIOUS Tumors

Aphthous ulcers Basal cell carcinoma

Behcet’s disease Squamous cell carcinoma

Crohn’s disease

Factitial disease

Fissures
The infectious ulcers are classically due to the STIs. The most common cause of genital ulcers in the world is herpes simplex. HSV in any Immunosuppressed patient can present with ulcers. These can be chronic, severe, punched out, and widespread. These are typically seen in a HIV positive individual. The other conditions are syphilis, chancroid, granuloma inguinale and rarely lymphogranuloma venereum. These conditions are all quite uncommon in North America.

Much more common are the non-infectious ulcers, particularly aphthae, which classically present as punched out, painful ulcers. They are mostly idiopathic but they can be associated with underlying conditions, see below. Aphthous ulcers are also seen in Behcet’s disease, Crohn’s disease and HIV. Crohn’s disease may present with the deep classic “knife-cut” type ulcers. Pyoderma gangrenosum can cause ulcers. Last in this group are the factitial ulcerations. Tumors, classically squamous cell carcinoma, also ulcerate.
The limitation to this classification is the possibility of missing the less common conditions that could cause vulvar ulcers and erosions such as drugs, irritant contact dermatitis, secondary infected bullous diseases etc.
2. Etiologic classification vulvar ulcers and erosions:
INFECTIONS

a) Venereal b) Non-venereal

Herpes simplex Candida Pseudomonas

Chancroid Herpes zoster Histoplasmosis

Granuloma Inguinale Varicella Cryptococosis

Lymphogranuloma venereum Hand Foot Mouth disease Tuberculosis

Syphilis Staph & Strep, Actinomycosis

Human Immunodeficiency Virus Typhoid ¶typhoid, Leishmaniasis

Brucellosis Schistosomiasis

Diphtheria, Amebiasis

Pseudomonas Epstein-Barr and Mycoplasma pneumoniae




Non-Bullous

Dermatoses

Bullous Dermatoses

Premalignant and Malignant Tumors

Infections


Miscellaneous


Irritant contact dermatitis

Drug Reaction*

Fixed Drug Rxn

LE

Crohn’s



Darier’s

Behcet’s


Pyo. gangrenosum

Hidr. suppurativa

Necrolytic Migratory Erythema


a) Autoimmune

BMM Pemphigoid

P. vulgaris

Bullous pemphigoid

Linear IgA Disease

EB Acquisita


b) Non-autoimmune

TEN / EM


Contact Dermatitis

Hailey-Hailey

EB Inherited


Premalignant and Malignant Tumors

VIN


BCC

SCC
Extramammary Paget’s Disease


Verrucous Carcinoma
Melanoma

Lymphoma


Leukemia

Hodgkins


Langerhans cell

histiocytosis



H. zoster

Varicella

Vaccinia

Hand/Foot/Mouth

Staph & Strep

Typhoid


Paratyphoid

Brucellosis

Diphtheria

Pseudomonas

Tuberculosis

Histoplasmosis

Actinomycosis

Cryptococcosis

Leishmaniasis

Schistosomiasis

Amebiasis


Rheumatoid

nodule


Gangrene

Acrodermatitis

Lymphangiectasis

Graft vs. Host

Spider bite

Hymenal Fissures

Reiter’s Disease
Wegener’s Granulomatosis
Factitial
Female Genital Mutilation

*12 meds for known to cause a drug reaction


Antibiotics

Sulfa


PCN (not as much trouble as before (no polymers attached)

cephalosporins


Cardiovascular

HCTZ


Lasix

Beta blockers

Ace inihibitors

Dilantin
Miscellaneous

Allopurinol

Vaccines


New biologicals

NSAIDs
Of all this list, the most important causes of ulcers and erosions are, in North America are:



Infections

Venereal Non Venereal

HSV Candida

Syphilis EBV

HIV M Pneumoniae



Dermatoses

Bullous Non-Bullous

Contact dermatitis Aphthosis

LS

LP

Drug



Contact

Crohn’s


Tumors SCC


APHTHAE (aphthous ulcers)

Canker sores on the vulva

Very common in the mouth and not uncommon on the vulva

Acute painful ulcer or ulcers of sudden onset

Minor or major in size, single or multiple
Clinical:

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

Often past history of oral aphthae – canker sores

Not Behcet’s

Associated with oral aphthae – complex aphthae

Acute (more common)

– usually a prodrome - fever, headache, malaise, GI upset

- EBV, Mycoplasma pneumoniae, viral upper respiratory infection or gastroenteritis, influenza, Strep

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
Complex Aphthosis:

Recurrent oral plus genital ulcers without Behcet’s

Site – usually on vulvar trigone or labia but can be into lower vagina

May scar – variable
For chronic or recurrent aphthae / complex aphthae look for:

Bowel disease - Crohn’s, Ulcerative colitis, Celiac disease

Infections – HIV

Behcet’s disease

Medications – cytotoxic, NSAIDs

Myeloproliferative disease, cyclic neutropenia, lymphopenia

Syndromes : rare

Sweet’s syndrome

Mouth and Genital Ulcers Inflamed Cartilage - MAGIC Syndrome

Periodic Fever, Aphthae, Pharyngitis, Adenitis - PFAPA Syndrome


Note Acute aphthae are probably immune complex related and can be precipitated by infection such as a viral illness. e.g. viral gastroenteritis or upper respiratory tract infection, influenza, CMV. Epstein Barr virus ( EBV) could directly infect the skin or cause an immune complex reaction. Mycoplasma pneumoniae can do the same. Streptococcal infection has been found. Most common cause of acute onset aphthae in a 12-20 year old is probably an infection.

For recurrent aphthae and complex aphthosis look for inflammatory bowel disease or, less likely, a lymphoproliferative problem.
Classification of Vulvar Aphthae:

Local Acute Genital Aphthosis or Complex Aphthosis
Local - can be associated with acute infections:

In young patients

Infections – viral gastroenteritis or upper respiratory tract infection, influenza, EBV, Mycoplasma pneumoniae, Streptococcal infection. Rare - CMV, typhoid, paratyphoid,Yersinia
Complex Aphthosis are recurrent aphthae oral and genital or recurrent severe genital aphthae
Diagnosis of exclusion

Cultures negative, biopsies non-specific and

blood work non-contributory
Differential diagnosis:

- HSV, Syphilis, HIV, Chancroid, LGV, Granuloma Inguinale

- pyoderma gangrenosum

- trauma


- contact dermatitis

Evaluation of Vulvar Aphthae:

Thorough history and physicaleye, 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 titer

Tests as indicated for – paratyphoid and typhoid (stool, blood culture), TB enterocolitis, Yersinia

GI investigations

for inflammatory bowel disease and celiac disease

Note – in HIV + patients with genital ulcers - 60% of genital ulcers

are due to aphthae and 40% to HSV


Treatment:

Pain control – topical – 5% lidocaine ointment

- systemic – mild, moderate pain – NSAID severe - opioids

Immunosuppression -

Prednisone 40 – 60 mg each morning until pain resolves (3-5 days, then ½ dose 3-5 days) with food

Methylprednisolone (Medrol) 4-8mg bid-tid 3-5 days then ½ dose 3-5 days) with food

Clobetasol or halobetasol 0.05% ointment AM & PM

Educate -Most often a one-time event, can recur


For persistent or chronic aphthae:

Oral corticosteroid for initial control - prednisone or methylprednisolone

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

thalidomide 100-150 mg per day

Prognosis:

Most often a one-time event

Scarring can occur

Occasionally recurrent

35% have a history of recurrence in usually older patients

References

Ulcers
Lewis FM, Velangi SS. An overview of vulvar ulceration. Clinical Obstetrics and Gynecology 2005;48:824-37.

Behçet’s Disease
Behçet H. Ueber rezidiverende, aphtoese, durch ein Virus veru sachte Geschwuere am mund, am Auge, und an den Genitalien. Dermatologische Wochenschrift 1937;105:1152-7.
Bills G, Kaufman RH, Bornstein J, Haefner H. Clinical question: ask the experts. Recurring painful vulvar ulcers. Journal of Lower Genital Tract Disease. 2005;9(1):55-8.
Haidopoulos D, Rodolakis A, Stefanidis K, Blachos G, Sotiropoulou M, Diakomanolis E. Behcet's disease: part of the differential diagnosis of the ulcerative vulva. Clinical & Experimental Obstetrics & Gynecology. 2002;29(3):219-21.
www.medscape.com/viewarticle/444060

MacCormack M, Phillips T. Behçet’s Disease: A clinical review. Wounds 2002;14:275-83.


Aphthous Ulcers
Huppert JS. Gerber MA. Deitch HR. Mortensen JE. Staat MA. Adams Hillard PJ. Vulvar ulcers in young females: a manifestation of aphthosis. Journal of Pediatric & Adolescent Gynecology. 19(3):195-204, 2006 Jun.
Farhi D. Wendling J. Molinari E. Raynal J. Carcelain G. Morand P. Avril MF. Frances C. Rozenberg F. Pelisse M. Dupin N. Non-sexually related acute genital ulcers in 13 pubertal girls: a clinical and microbiological study.2009;145;38-45
Lehman JS. Bruce AJ. Wetter DA. Ferguson SB. Rogers RS. Reactive nonsexually related cute genital ulcers: Review of cases evaluated at May Clinic. , J Am Acad Dermatol. 2010 Jul;63(1):44-51.
Herpes

Corey L, Wald A, Patel R et al. Once-daily valacyclovir to reduce the risk of transmission of genital herpes. New England Journal of Medicine 2004;350:11-20,67-8.


Freedman JM, Gacicia K. Management and treatment of genital herpes. A practical approach to patient care. Advance for Nurse Practitioners. 2002;10(4):71-4, 80.
Richwald GA, Warren TJ. The diagnosis and management of genital herpes. The silent epidemic. www.medscape.com/viewprogram/1990
Roberts CM. Genital herpes. Evolving epidemiology and current management. JAAPA/Journal of the American Academy of Physician Assistants. 2003;16(2):36-40.
Steben M. Genital herpes simplex virus. Clinical Obstetrics and Gynecology. 2005;48:838-44.
Yeung-Yue KA, Brentjens MH, Lee PC, Tyring SK. Herpes simplex viruses 1 and 2. Dermatologic Clinics. 2002;20(2):249-66.







The database is protected by copyright ©dentisty.org 2016
send message

    Main page