By ProPath Staff
Inflammatory diseases of the vulva can pose a diagnostic and therapeutic challenge. Understanding the basic disease categories and the role of biopsy can facilitate management of these conditions. Vulvar dermatoses can be broadly divided into four groups (based on clinical and microscopic features).1 Table 1.
Spongiotic dermatitis is the histologic term for “eczema”, which is a common cause of vulvar symptoms, i.e. itching, stinging, or burning. Eczema frequently results from the use of topically applied products such as medicaments or fragrances. Contact dermatitis can be either nonspecific (irritant) or can be a true, immune- mediated allergy. Atopic and seborrheic dermatitis are other causes of spongiosis. On exam, poorly demarcated erythema, scaling and erosions are observed. Treatment includes gentle hygiene with strict avoidance of all products and a limited course of topical steroids. In recalcitrant cases, patch testing for cutaneous allergy might be helpful.
Lichen sclerosus (LS), a sclerosing disorder, presents as erythematous (early) and porcelain white (later) sclerotic plaques, which range from asymptomatic to intensely pruritic. Early intervention with an ultrapotent topical steroid is necessary to prevent permanent sclerosis. Because of an increased risk for squamous cell carcinoma (SCC), these patients require ongoing therapy and surveillance.
Papulosquamous dermatitis can be intensely pruritc, as in lichen planus (LP) or relatively asymptomatic, as in psoriasis. Physical findings include more sharply demarcated plaques, and there is often extra-genital skin involvement. Routine therapy includes topical steroids. A severe variant of ulcerative LP, the gingivovulvovaginal syndrome, is treated aggressively with systemic immunosuppressants. Patients with LP are also at increased risk for SCC.
Immunobullous diseases, which result from deposition of immunoreactants in the epithelium, lead to clinically apparent erythema, blisters or erosions. ome of these conditions, if untreated, are disabling and result in extensive scarring. Definitive diagnosis of these disorders requires a separate (perilesional) iopsy for direct immunofluorescence, which allows for visualization of the deposited antibodies. Systemic corticosteroids or other immunosuppressants are often utilized in treatment.
Biopsy considerations
The initial indication for performing a vulvar biopsy might be to exclude dysplasia or malignancy. Even in the setting of known HPV, concurrent dermatitis is usually the cause of clinical symptoms (burning or pruritus).2 The diagnosis and proper classification of a dermatosis will assist in management decisions. Further, early diagnosis of potentially scarring conditions is important, as is the recognition of an increased cancer risk in lichen sclerosus and lichen planus.
Accurate microscopic diagnosis of inflammatory conditions requires visualization of the dermis/lamina propria; accordingly, a punch biopsy specimen (with a minimum diameter of 3 mm) is optimal. Care is taken not to crush the specimen with forceps, and the tissue is placed immediately into formalin. In suspected immunobullous disease, a second biopsy from perilesional skin is obtained and placed into a special transport medium (e.g. Michel’s) for direct immunofluorescence testing.
Communication between the clinician and dermatopathologist can be integral to arriving at an accurate diagnosis. Information on the requisition sheet such as the specific biopsy site (e.g. “labia minora” rather than “vulva”), a brief description of signs and symptoms, and clinical impression, all provide valuable clues to the pathologist. In addition, telephone communication with the pathologist can sometimes be helpful to clarify the report and to address any of the clinician’s questions or concerns.
References:
1.Hammock LA, Barrett TL. Inflammatory dermatoses of the vulva. J Cutan Pathol. 2005 Oct;32(9):604-11. Review. PubMed PMID: 16176297.
2.Fischer G, Spurrett B, Fischer A. The chronically symptomatic vulva: aetiology and management. Br J Obstet Gynaecol. 1995 Oct;102(10):773-9. PubMed PMID: 7547732.