Inflammatory Dermatoses Eczema

Vulvar eczema is characterized by erythema and scaling on the outer labia majora and erythema and fissures and erosions on the inner aspects. Vulvar eczema is pruritic, often out of proportion to the clinical findings. The skin may become ede-matous, excoriated, and lichenified because of scratching.

Irritant Contact Eczema

This is the commonest form of vulvar eczema in clinical practice. This is usually seen in the elderly and is often because of urinary incontinence. It is also more likely to occur in fair-skinned individuals who have sensitive skin at other sites as well (Fig. 1).

Allergic Contact Eczema

Allergic contact eczema is a Type IV cell-mediated allergic reaction that takes 48 to 72 hours to develop following exposure to the offending allergen. This type of allergy is uncommon on the vulvar skin alone and is likelier to be found if there is perianal eczema as well. The commonest allergens include topical antibiotics and preservatives in the ointments or creams that are being used. The topical steroid itself can on rare occasions be responsible. It is important to patch test the patient if the vulvar rash is worsening with treatment.

Seborrheic Eczema

This is the second most common type of vulvar eczema that tends to affect other flex-ural sites including the axillae, submammary, and inguinal folds. There may also be scalp involvement. On occasions it may be very difficult to differentiate this form of eczema from flexural psoriasis; however, with the former there should be no nail involvement (Fig. 2).

Exema Exposed
Figure 1 (See color insert) Irritant eczema. Glazed erythema of the most exposed areas in a patient with urinary incontinence.

Atopic Eczema

This does not tend to occur on the vulvar skin alone and the vulvar eczema that is associated with atopy is usually an irritant eczema, because the skin of atopic individuals has a lower threshold for irritant reactions.

Treatment of all types of eczema includes the avoidance of irritants such as bubble baths and shampoos and the use of a soap substitute. A mild-to-moderately potent topical corticosteroid ointment will help the inflammation and relieve the itch. Barrier ointments are helpful if the patient is incontinent (Table 1).

Itchy Labia Majora
Figure 2 Seborrhoeic eczema. Orange red erythema of the labia majora area extending into the genitocrural folds and perianal area.
Table 1 Eczema

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis

Therapy

Eczema

Itch, pain if

Ill-defined

Psoriasis and

Soap substitute,

fissured,

erythema

Hailey-Hailey

emollients, and

excoriated,

mainly on labia

mild to

and eroded

majora and

moderate

minora with

topical steroid

edema and

lichenification

The anogenital skin can be involved as part of generalized psoriasis but the form more characteristically seen on the vulva is flexural psoriasis. The erythema is well defined and there may be scale on the hair-bearing parts, i.e., the mons pubis and outer labia majora (Fig. 3A and B). The inner aspects of the labia majora tend to be red and fissured and nonscaly because the environment is damp and occluded. The labia minora are red and edematous. It is helpful to look at other sites to confirm the diagnosis of flexural psoriasis, e.g., scalp, axillae, submammary areas, periumbilical, inguinal, genitocrural folds, and natal cleft. In addition, there may be dystrophic nail changes with pitting and subungual hyperkeratosis.

The main complaint is itch and often there is also a burning sensation. On examination there is well-defined erythema, particularly in the interlabial sulci with fissuring and a buildup of keratinous debris. The changes may extend to involve the perianal area and the natal cleft.

Treatment. A soap substitute, i.e., emulsifying ointment or aqueous cream and a moderately potent topical corticosteroid, e.g., betamethasone once a day. A barrier ointment, e.g., zinc ointment may also help (Table 2).

Introital Margin Figure
Figure 3 (See color insert) (A) Flexural psoriasis. Well-marginated erythema of anogenital skin with some crusting. (B) Psoriasis. Characteristic elongated rete ridges and papillae, dilated vessels within papillae, and intraepithelial (Munro's) microabscess.
Table 2 Psoriasis

Clinical

Differential

Diagnosis

Symptoms

appearance

diagnosis

Therapy

Psoriasis

Itch, burning

Beefy red and

Eczema. Lichen

Soap substitute,

pain if fissured

well-defined

planus,

emollients, and

or eroded

erythema

Reiter's

moderate to

disease, and

potent topical

extramammary

steroid; barrier

Paget's

ointments

Reiter's Disease (Reiter's Syndrome and Circinate Ulcerative Vulvitis)

This condition is characterized by a nonsuppurative polyarthritis frequently accompanied by urethritis, cervicitis, and conjunctivitis. Reiter's disease is commoner in males and it may be triggered by an enteric or a lower genital tract infection. There seems to be a predisposition in those individuals who are positive for the human leukocyte antigen B27. The cutaneous manifestations probably represent a particular form of psoriasis. Circinate balanitis is well recognized. The corresponding vulvitis is much rarer but has been described (1). The lesions that occur can be eroded, ulcerative, or scaly.

Histologically, the changes are of those seen in pustular psoriasis with hyper-keratosis, parakeratosis, psoriasiform hyperplasia, an absent granular layer, and collections of polymorphs in the epidermis (2). Treatment is the same as for psoriasis (see previous section).

Lichen Planus

Lichen planus (LP) can affect the anogenital skin or vagina in isolation or at the same time as a generalized outbreak, when about 20% of patients will have genital lesions (3). The vulvar lesions are similar to those seen on nongenital skin sites and may be violaceous or erythematous papules, white or annular plaques, and erosions with or without a lacy white border. Sometimes, the main findings are erosions and ulcerations. If the LP is confined to the vulva and/or vagina, the lesions are more likely to be erosive (Fig. 4). Examination of the oral mucosa is helpful as LP may be found on the tongue (Fig. 5), palate, gingiva, and lateral buccal mucosa. There is a small risk (> 4%) of squamous cell carcinoma (SCC) arising on a background of LP. The other clinical forms of vulvar LP include

Pigmented Flexural LP

The lesions are characteristically distributed in the inguinal and genitocrural folds. The individual lesions are brown pigmented patches, some so deeply pigmented that they resemble melanocytic nevi. Early lesions may have a violaceous erythematous appearance. This form of LP will also be found in the other flexural sites particularly the inframammary areas and axillae.

Vulvo-Vaginal-Gingival LP (Syndrome of Hewitt and Pelisse) This is a distinctive erosive form of LP that is clinically very similar to mucous membrane pemphigoid (4,5). In the past, many cases labeled desquamative vaginitis were

Desquamative Inflammatory Vaginitis

Figure 4 Lichen planus. Eroded areas on a background of pallor. The architecture remains well preserved.

probably this entity. Vulvo-vaginal-gingival LP principally affects the inner aspects of the labia minora, vestibule, and vagina; the lesions are painful and itchy. Patients will complain of dyspareunia, dysuria and, if there is vaginal involvement, an increased vaginal discharge, and postcoital bleeding. Clinically, the vulvar lesions are eroded and have a distinctive fine white lacy border. The anal margin may also

Figure 5 Lichen planus on the dorsal aspect of the tongue.

Young Labia Minora

Figure 6 (See color insert) Vulvovaginal lichen planus. Glazed erythema on the inner aspects of the labia minora bordered on the left-hand side with a white edge.

be involved. The vaginal lesions are velvety red erosions or bright red glazed erythema patches (Fig. 6), which when touched are friable and bleed. Vaginal syne-chiae and adhesions occasionally develop, leading to vaginal stenosis. The cervix may also be involved. The oral involvement is a glassy red erythema of the gingivae (Fig. 7), but more typical lesions of LP can be found on the tongue or lateral buccal mucosae. Very unusual sites of involvement have been described on the conjunctiva (6), lachrymal gland canal (7), esophagus (8), and the auditory canal (9).

Lichen Planopilaris

This is a very rare variant that affects the hair-bearing skin of the vulva (10).

Hypertrophic LP

This variant is often the most difficult to diagnose and treat. The clinical appearance is of sheets of hyperkeratotic whitened epithelium that encase the vulva and often extend perianally. There is usually a loss of the labia minora with introital narrowing and the clitoris may be buried (Fig. 8A). The disease is often so long standing that the typical histological features of LP are not found. The diagnosis is confirmed by a biopsy and the typical changes are irregular acanthosis with a saw-tooth appearance of the rete pegs, an increased granular layer, and disruption of the basal layer with a

Figure 7 (See color insert) Lichen planus, sulcus.

Glazed erythema of the gingiva with striae in the

Vulvar Eczema

Figure 8 (A) (See color insert) Hypertrophic lichen planus (LP). There is loss of the labia minora and the clitoris is buried. The vulval skin is white and hyperkeratotic with erythema in the inner aspect. (B) LP—characteristic band-like infiltrate of chronic inflammatory cells in the dermis, sawtooth deformity of the rete ridges, and hyperkeratosis. (C) (See color insert) LP—saw-toothing deformity of rete ridges, uneven hypergranulosis, and orthokeratotic hyperkeratosis.

Figure 8 (A) (See color insert) Hypertrophic lichen planus (LP). There is loss of the labia minora and the clitoris is buried. The vulval skin is white and hyperkeratotic with erythema in the inner aspect. (B) LP—characteristic band-like infiltrate of chronic inflammatory cells in the dermis, sawtooth deformity of the rete ridges, and hyperkeratosis. (C) (See color insert) LP—saw-toothing deformity of rete ridges, uneven hypergranulosis, and orthokeratotic hyperkeratosis.

closely apposed dermal band-like lymphocytic infiltrate (Fig. 8B and C). The acanthosis and hyperkeratosis are marked in the hypertrophic form and because of the chronicity of this form the characteristic band-like infiltrate is not obvious but will be found focally. Eosinophilic colloid bodies may be seen. Immunofluores-cent (IMF) studies will reveal uneven fibrinogen staining of the basement membrane and IgM cytoid bodies. These studies also help to differentiate LP from the other blistering diseases, particularly mucous membrane pemphigoid.

The main differential diagnosis is usually lichen sclerosus (LS) but mucous membrane pemphigoid, pemphigus, lupus erythematosus, and morphea could also be included. In some cases the differentiation between LS and LP can be extremely difficult and a diagnosis depends on a combination of clinical, histological, and IMF

findings and response to treatment. Frequently, there are cases of LP misdiagnosed as Zoon's vulvitis.

Treatment: First-line treatment is with potent topical steroids. If the vagina is involved, topical steroids can be introduced with a vaginal applicator, or steroid foams or suppositories that are available for inflammatory bowel conditions can be used (11). Topical retinoids are useful for hyperkeratotic and hypertrophic LP and recently topical tacrolimus has been used (12). Other treatments tried with variable success include oral steroids, oral and topical retinoid, methotrexate (13), cyclosporin, and azathioprine (Table 3).

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