Behets Syndrome

This is a syndrome of recurrent oral ulceration accompanied by two of the following: genital ulceration, eye lesions including anterior uveitis and retinitis, other cutaneous lesions, i.e., erythema nodosum, sterile pustules, erythema multiforme, and pustu-lation at sites of trauma (pathergy). It is a multisystem disorder (37) and there may be gastrointestinal, neurological, psychiatric, rheumatological, cardiological, and pulmonary involvement. Vascular complications include superficial and deep vein thromboses, arterial occlusions, and aneurysms (Fig. 16). Vulvar ulcers occur less frequently than the oral ulceration and they are clinically similar to benign aphthae although they may be larger and or more numerous and tend to last longer. They heal with scarring. Diagnosis is sometimes difficult and often only made as the clinical signs and complications evolve over a period of time. The geographic distribution of cases suggests either local endemic agent or physician overacceptance of the diagnosis.

Treatment: Topical steroids with or without a topical antibiotic can be used for minor vulvar ulceration. Severe ulceration may require an oral steroid or colchicine and immunosuppressants. Thalidomide is reserved for recalcitrant disease.

Vasculitis Disease Table
Figure 16 Behcet's disease—the lesions are characterized by vasculitis and perivasculitis deep to the ulcerations.

Table 10 Behcet's Syndrome

Clinical Differential

Diagnosis Symptoms appearance diagnosis Therapy

Behcet's Pain Ulceration and Aphthae Thalidomide pustulation Potent topical steroids with or without a topical antibiotic, oral steroids, and colchicine

It is important to have an eye assessment, because ocular complications are common. Anticoagulants are indicated for thrombotic disease. It is important to have a multidisciplinary team involved in the management of patients with Behcet's syndrome (Table 10).

EROSIVE, ULCERATIVE, AND BULLOUS DERMATOSES Chronic Ulcerative and Bullous Diseases

Any chronic solitary ulcer of the genital mucosa must be considered malignant or infective until proven otherwise. Generalized ulceration of the vulva can be because of an underlying bullous condition but because of the friction and forces at the site, blisters do not often remain intact and erosions and ulcers are seen instead. The differential diagnosis of chronic ulceration can come under any of the headings listed below.

Vaginal Darier Disease
Figure 17 (See color insert) Hailey-Hailey disease. There is erythema with maceration in the genitocrural fold and tiny erosions.

Genetic

Congenital epidermolysis bullosa is a rare disorder of the skin, which is characterized by blistering and scarring of the skin in response to friction or trauma. The two types that involve the vulvar and vaginal area are dystrophic and functional epider-molysis bullosa.

Hailey-Hailey disease (benign familial pemphigus) and Darier's disease may also be responsible for chronic recurring erosions and ulceration of the vulvar skin and genitocrural folds (Fig. 17). Sometimes turbid intact vesicles are seen. The diagnostic histological finding is acantholysis of the epidermal cells. Hailey-Hailey and Darier's disease affect other sites as well as the vulva. Darier's disease is characterized by notching of the nails and white longitudinal lines. Hailey-Hailey disease does have the white lines but not the notching but vaginal lesions have been described in Hailey-Hailey diseases but not in Darier's disease. There appears to be a distinct clinicopathological entity, genital papular acantholytic dyskeratosis, that affects the vulvar area only (38,39). It may be a forme fruste of Hailey-Hailey or Darier's disease. Exacerbations may follow friction, infection, irritants, and herpes simplex infection.

External Trauma

Nymphohymeneal Tear. This is usually because of a tear around the nympho-hymeneal sulcus and most frequently occurs after sexual intercourse (Fig. 18). The tear will heal spontaneously but can recur. These tears tend to occur around the nymphohymeneal sulcus usually in the posterior segment in the 5 and 7 o'clock positions. Those that do not heal can be excised radially. Recurrent superficial tears at the fourchette may sometimes occur in celibate women.

Posterior Fourchette Lesion

Figure 18 Nymhohymenal tear healing at 7 o'clock in the inner aspect of the vestibule and a fresh lesion just above at 8 o'clock.

Posterior Fourchette Tear
Figure 19 (See color insert) Erythema multiforme—characteristic targetoid lesions. Source: Image courtesy of W. Clark Lambert, MD, PhD., UMDNJ-New Jersey Medical School, Newark, New Jersey, U.S.A.
Pyoderma Gangrenosum Female Genitals
Figure 20 Stevens-Johnson syndrome. This superficial biopsy of a blister shows the characteristic epidermal necrosis.

Figure 21 Pyoderma gangrenosum—characteristic ulcer and heavy inflammatory infiltrate.

Dermatitis artefacta and radiation damage following treatment of a gynecological malignancy may also be a cause of chronic ulceration.

Drugs

These are slightly unusual to be the sole cause of a problem in the anogenital area alone and genital involvement occurs as part of a generalized skin eruption.

Erythema multiforme may produce vulvar ulcers and erosions. The etiology is often herpes simplex but many are drug induced. The lesions are usually aphthous-like ulcers and there is usually oral involvement at the same time. The typical target-like lesions may be seen at other nongenital sites (Fig. 19).

Stevens-Johnson syndrome (Fig. 20) and toxic epidermal necrosis is a severe problem and is often drug induced. Some of the drugs involved include phenytoin, trimethoprim, sulphonamides, and nonsteroidal anti-inflammatories. The onset is acute with systemic symptoms. Shallow ulcers, erosions, and flaccid

Bullous Pemphigoid Vulva
Figure 22 (A) (See color insert) Bullous pemphigoid. Erythematous lesions with erosions and flaccid blisters. (B) Pemphigoid—characteristic subepidermal bulla and eosinophilic-rich dermal infiltrate.
Vulvitis Pictures
Figure 23 Pemphigus. (A) (See color insert) Erosions on right side of vulva. (B) Characteristic intraepidermal bulla, most prominent in the deeper aspect of the epithelium.

blisters develop on the inner aspects of the vulva and vagina. This may rapidly lead to adhesions and vaginal stenosis. The other mucous membranes are similarly affected, particularly the eyes and mouth. The lesions on the nonmucosal sites start as an indurated erythema and then blister, leaving large expanses of denuded skin.

Fixed drug eruption (FDE) may present as a bullous lesion (see below). Malignancy

The two commonest malignant ulcers of the vulva are SCC and basal cell carcinoma. Melanoma may also present as an amelanotic ulcerating nodule. Langerhans cell histiocytosis may present with vulvar and vaginal ulceration.

Infection

Chronic infective ulcers occur in tuberculosis, actinomycosis, and other deep mycoses. The late stages of lymphogranuloma venereum (chlamydia) cause ulceration and scarring. Patients who are immunosuppressed may have chronic ulceration with herpes simplex and cytomegalovirus.

Table 11 Lymphangiectasia

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis

Therapy

Lymphangiectasia

Itch, wetness

Shiny skin-

Lymphangioma

Potent topical

because of

colored

circumscripta

steroid

lymphorrhea

micropapules

ointment

or pseudo-

or CO2

vesicles

laser

Squamous Hyperplasia Vulvar
Figure 24 Lichen simplex (kichen simplex chronicus, squamous cell hyperplasia)—there is hyperkeratosis and acanthosis with fusing of rete ridges.

Inflammatory

LS, LP, and lupus erythematosus can all cause chronic ulceration. Vulvar and vaginal lesions are very rare in systemic lupus erythematosus. Hidradenitis suppurativa and Crohn's disease can sometimes be difficult to distinguish and they can occur together.

Pyoderma gangrenosum (Fig. 21) may occur in association with inflammatory bowel disease, rheumatoid arthritis, and hematological malignancies. Vulvar pyoderma gangrenosum can often accompany Crohn's disease and it can be difficult differentiating this from a fistulous tract from the bowel. The lesion may be solitary and large or occasionally multiple and small. The initial lesion may be a hemorrhagic vesicle or bulla.

Autoimmune Bullous Diseases

The mucocutaneous autoimmune blistering disorders can affect the vulva and vagina. The integrity of the cellular adhesion of the epidermal cells and/or the basement membrane zone is damaged by the antibody attack and the various blistering diseases that occur are classified according to the site of the target antigen. Several of the autoimmune bullous diseases' effect can affect the vulva. Pemphigoid (Fig. 22A and B), linear IgA disease, and pemphigus (Fig. 23A and B) may all present with vulvar and vaginal ulceration.

Treatment: The underlying cause, be it drug, dermatosis, or infection needs to be identified and managed. Microbiological studies to exclude bacterial or viral infection must be carried out and a detailed drug history taken, not forgetting over-the-counter medicines. General measures include potassium permanganate 1:10,000 soaks or Burow's solution, topical antibacterial such as silver sulfadiazine, and oral or topical analgesics.

Table 12 Lichen Simplex

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis

Therapy

Lichen

Severe itch

Pale lichenified skin;

Eczema

Potent topical

simplex

no scarring; usually

steroid

unilateral

Fluconazole Topical Solution
Figure 25 (A and B) Indurated erythematous and discoid lesions of a fixed drug eruption because of fluconazole. (C) (See color insert) Fixed drug eruption—vacuolization of the basal layer and pigmentary incontinence are seen.

MISCELLANEOUS Lymphangiectasia

Vulvar lymphangiectasia is a result of dilatation of superficial lymphatics secondary to obstruction of previously normal deep lymphatics (40). It is most frequently seen

Postinflammatory Hyperpigmentation
Figure 26 (See color insert) Postinflammatory hyperpigmentation—pigment incontinence is seen.

Table 13 Fixed Drug Eruption

Differential

Diagnosis Symptoms Clinical appearance diagnosis Therapy

Fixed drug Itch Usually solitary urticated Lichen planus; Stop the eruption erythematous patch that dermatitis medication leaves an area of artefacta and hyperpigmentation; erythema occasionally multiple multiforme lesions; can be bullous following surgery or radiation for vaginal or cervical malignancy. It may also be a feature of Crohn's disease (Figs. 9 and 10).

Treatment: This is sometimes required if there is marked lymphorrhea, leaving the patient permanently wet, and CO2 laser can give long-term symptomatic relief (41) (Table 11).

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