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Plasma cell vulvitis and response to topical steroids: A case report

Posted on: Saturday, 23 August 2003, 06:00 CDT

Summary: Plasma cell vulvitis is a condition which, due to its rarity, can be difficult to diagnose and challenging to treat. This report describes a case of plasma cell vulvitis, its response to topical steroids and summarizes what is known about the condition.

Keywords: vulvitis, plasma cell, clobetasol

Introduction

Plasma cell vulvitis is an extremely rare skin condition characterized by plasma cell infiltration. In our report, we discuss a patient with classical signs and symptoms of this condition, which was misdiagnosed initially as genital herpes. In our patient potent topical steroid is shown to be an effective treatment.

Case report

A 55-year-old woman was referred to the genitourinary clinic by her general practitioner with a four-month history of vulval pain, itching, a stinging sensation and labial ulcers. Her symptoms had not improved with topical and oral anti-herpetic therapy. There were no other genitourinary symptoms, no other skin abnormalities and no relevant past medical history.

On examination, she had patchy areas of bright red moist skin on the vulva, which were covered in slough. These areas were surrounded with oedema and whitened areas of skin. There were two areas of superficial skin erosion, 1-2 cm in diameter (Figure 1) which bled on contact.

Screening for sexually transmitted infections was negative. Two skin biopsies were taken, one from the eroded area on the labia majorum and the other from the erythematous area. One of the biopsies showed ulcerated skin and the other one showed thin squamous epithelium. There was an inflammatory cell infiltrate composed predominantly of plasma cells and haemosiderin deposits in the upper dermis. The report stated that these findings were consistent with plasma cell vulvitis. Clobetasol propionate cream twice daily was prescribed.

Four weeks later, the patient was reviewed at clinic. She was asymptomatic, and on examination, the vulval skin looked markedly improved (Figure 2). She had been using clobetasol cream once daily for two weeks. Advice was given to reduce the cream usage to once a week.

Figure 1. The vulva showing patchy vulvitis with superficial skin erosion

Figure 2. Vulvitis markedly improved after four weeks of local steroid

At three-months' review there was no recurrence of her symptoms and mild erythema was found on examination.

Discussion

Plasma cell vulvitis (or vulvitis circumscripta plasmacellularis or Zoon's vulvitis) is a chronic inflammatory skin condition. In contrast to plasma cell balanitis, it is very rare - there have been only 31 cases reported world-wide. It affects women aged 8-80 years of age1,5. It causes red macular lesions anywhere in the vulva with pinpoint darker spots. There may also be erosions. These lesions may be asymptomatic or cause itching, burning, dyspareunia, dysuria and bleeding1-4.

The specific cause of this condition is unknown. Suggested predisposing factors include warmth, friction, poor hygiene, herpes simplex infection and other chronic infection1,2. The differential diagnosis includes squamous cell carcinoma, candida, herpes simplex, lichen planus, pemphigus vulgaris, trauma, fixed drug eruptions and contact dermatitis1-3.

Skin biopsy shows the typical histological features of plasma cell vulvitis. These include epithelial thinning due to decreased size and quantity of keratocytes and the loss of granular and horny cell layers. The keratocytes are described as being 'diamond shaped'. The intracellular spaces increase, possibly due to tissue oedema. There is a preponderance of plasma cells, which often account for over 50% of cells present. There is vascular proliferation and dilatation. Haemosiderin deposition gives the lesions their characteristic colour. There are no premalignant changes1,2.

The cases of plasma cell vulvitis described in the literature have been treated with various different therapies. Topical steroid creams and intralesional injections have been used with varying degrees of success1-4. Retinoid therapy and interferon have shown benefit in a few patients. Other methods tried included antifungal and antibiotic preparations, caudal nerve blocks, cryotherapy and simple excision3,4. Our patient responded well to topical steroid therapy.

International Journal of STD & AIDS 2003; 14: 568-569

References

1 McCreedy CA, Melski JW. Vulvar erythema. Arch Dermatol 1990;126:1351-6

2 Kavanagh GM, Burton PA, Kennedy CTC. Vulvitis circumscripta plasmacellularis (Zoon's vulvitis). Br J Dermatol 1993;129:92-3

3 Morioka S, Nakajima S, Yaguchi H, et al. Vulvitis circumscripta plasmacellularis treated successfully with interferon alpha. J Am Acad Dermatol 1988;19:947-50

4 Robinson JB, Im DD, Simmons-O'Brien E, Rosenshein NB. Etretinate: therapy for plasma cell vulvitis. Obstet Gynecol 1998;92:706

5 Albers SE, Taylor G, Huyer D, Oliver G, Krafchik BR. Vulvitis circumscripta plasmacellularis mimicking child abuse. J Am Acad Dermatol 2000;42:1078-80

(Accepted 22 November 2002)

Loay David MBChB MRCP and Kate Massey MBChB

Department of Genitourinary Medicine, Shepperton House, George Eliot Hospital NHS Trust, College Street, Nuneaton CV10 7DJ, UK

Correspondence to: Dr L David

E-mail: loay.david@geh.nhs.uk

Copyright Royal Society of Medicine Press Ltd. Aug 2003

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