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6 Symptoms and Signs in Vulval Disease

Fiona M. Lewis

CHAPTER MENU

  Symptoms in vulval disease Vulval ulceration Vulval oedema Acute vulval oedema Chronic vulval oedema

  Signs in vulval disease

  References

Symptoms in vulval disease

Symptoms relating to vulval disorders tend to fall into a few clear categories. Itch, soreness, and pain are the common descriptions that women will give. It is always important to clarify exactly what the patient experiences when they report symptoms, as misunderstanding is easy. If a patient describes irritation, this does not always correlate with itch, and so asking them if they have the desire to scratch (which does define pruritus) is helpful. The same is true for signs. Patients may report ‘blisters’, but these are rarely, if ever, true bullae, which can then lead to unnecessary investigations.

As different specialties are involved in treating women with vulval disease, it is important to have a clear and common terminology for describing lesions. This should ensure that the same language is spoken when discussing cases with colleagues and in research.

There are classifications of disease according to clinical [1] and histological patterns [2]. These may be helpful initially, but they can be simplistic as some disorders can fit into more than one category and there can be atypical presentations of common disease.

Some common causes of vulval symptoms are shown in Table 6.1.

There are two specific situations where there is a more extensive differential diagnosis: vulval ulceration and vulval oedema. An approach to patients presenting with these symptoms is considered here, with more details on the specific conditions in the appropriate chapter.

Vulval ulceration

Patients presenting with one or more vulval ulcers can pose a diagnostic challenge [3]. Some clinical patterns of disease such as herpes simplex infection are easy to recognise. However, some chronic ulcers will require extensive further investigation in order to make a diagnosis.

There are four main causes of vulval ulcers:

 Infection

 Inflammation

 Malignancy

 Trauma

A full history must be taken, as outlined in Chapter 5. When examining the patient, the site, number, and characteristics of any ulcer should be noted. A differential diagnosis can then be formulated (Figure 6.1).

Vulval oedema

In a similar way, there is a wide differential diagnosis in patients who present with vulval swelling as the predominant symptom. The potential causes are shown in Figure 6.2.

Table 6.1 Common causes for vulval symptoms.

	Pruritus	Soreness/discomfort	Pain	Dyspareunia
Infection – sexually transmitted	Scabies Trichomonas vaginalis	Any vaginal discharge can cause vulval soreness	Herpes simplex	Herpes simplex
Infection – non‐sexually transmitted	Candidiasis Tinea cruris	Candidiasis	Herpes zoster	Candidiasis
Inflammatory	Eczema/lichen simplex Psoriasis Lichen sclerosus Lichen planus – classic or hypertrophic types	Erosive lichen planus Immune‐bullous disease Irritant dermatitis	Crohn’s disease Hidradenitis suppurativa	Erosive lichen planus Lichen sclerosus Psoriasis Immuno‐bullous disease Graft versus host disease
Malignancy	High grade squamous intraepithelial lesion (HSIL)	HSIL	Any malignant tumour	Extra‐mammary Paget’s disease Any malignant tumour
Neuropathic	Dysaesthesia for itch	Extra‐mammary Paget’s disease	Vulvodynia	Localised provoked vulvodynia
Others	Urticaria Syringomas	SJS/TEN Graft versus host disease	Acute reactive genital ulcers (Lipschutz) SJS/TEN Neuroma	Mechanical fissuring of fourchette or hymenal ring

Figure 6.1 Causes of vulval ulceration.

No classification for the types of oedema exists, but a useful way of thinking about the differential diagnosis is to consider acute and chronic causes.

Acute vulval oedema

A degree of oedema is often seen in patients with acute inflammatory conditions such as candidiasis or eczema. This settles with treatment of the primary problem. Urticaria or angio‐oedema, including hereditary angio‐oedema, may affect the vulva. Acute swelling will occur in type I allergic reactions (see Chapter 22). Vulval oedema has been reported in the ovarian hyperstimulation syndrome, a rare complication following ovulation in cases of infertility [4]. The mechanism was thought to be fluid retention, decreased oncotic, and increased hydrostatic pressure. Gross vulval oedema has been described in pre‐eclampsia [5] and vulval oedema occurring in pregnancy [6] and after delivery have been rarely reported [7].

Figure 6.2 Causes of vulval oedema.

Rarely, a direct passive transfer effect can also result in vulval oedema in patients undergoing peritoneal dialysis, in which the channel can be a small hernia or a defect of the peritoneal fascia [8]. Acute, but self‐limiting unilateral vulval oedema has also been described after instillation of adhesion barrier solution at laparoscopy [9].

Chronic vulval oedema

This topic is dealt with in Chapter 33.

Figure 6.3 Diagrammatic section of normal skin.

Signs in vulval disease

The keratinised epithelium has four layers which overlies the dermis containing adnexal structures and the vascular network (Figure 6.3). Changes will occur with disease processes which will manifest in different ways. It is important to be able to describe these lesions accurately. These are detailed in Table 6.2 and shown diagrammatically in Figure 6.4.

Figure 6.4 Features of cutaneous lesions.

Table 6.2 Types of cutaneous lesions.

Lesion	Description	Example
Papule	Small palpable lesion up to 5 mm in diameter	Syringoma
Macule	Visible lesion up to 5 mm in diameter but not palpable	Pityriasis versicolor
Nodule	Palpable lesion >5 mm in diameter	Malignancy
Plaque	Flat palpable lesion >5 mm in diameter	Psoriasis, HSIL, lichen sclerosus
Ulcer	Break in epithelium that reaches into dermis	Crohn’s disease, malignancy, etc.
Erosion	Superficial epithelial loss, not into dermis	Erosive lichen planus
Vesicle	Fluid‐filled lesion up to 5 mm in diameter	Herpes simplex
Bulla	Fluid‐filled lesion >5 mm	Bullous pemphigoid
Pustule	Pus‐filled lesion	Folliculitis
Fissure	Linear break in epithelium which can involve dermis if deep	Mechanical hymenal fissure, psoriasis, lichen sclerosus, Crohn’s disease
Comedone	Keratin plug open to surface	Hidradenitis suppurativa

References

1 1 Lynch, P.J., Moyal‐Barracco, M., Scurry, J. and Stockdale, C.2011 ISSVD Terminology and classification of vulvar dermatological disorders: An approach to clinical diagnosis. J Low Genit Tract Dis. 2012 Oct; 16(4): 339–344.

2 2 Lynch, P.J., Moyal‐Barracco, M., Bogliatto, F. et al. 2006 ISSVD classification of vulvar dermatoses: Pathologic subsets and their clinical correlates. J Reprod Med. 2007 Jan; 52(1): 3–9.

3 3 Bohl, T.J. Vulvar ulcers and erosions: A clinical approach. Clin Obstet Gynecol. 2015; 58: 492–502.