Concomitant lip and vulval involvement of Crohn's disease: A rare occurrence

Case report

A 20-year old female presented with diffuse vulval swelling for two years and lip swelling for four months. The lip swelling was insidious and associated with fissuring and angular cheilitis. The vulval swelling was also insidious in onset, initially in the midline and gradually progressing to involve both labia. There was no colorectal involvement. The patient was known to have Crohn's disease, and was on treatment. There was no history of fever, vaginal bleeding, inguinal swelling, genital ulcer or high-risk sexual behaviour.

On clinical examination, diffuse vulval swelling with erythematous indurated labia majora and clitoris with erosions (Fig. 1(A)) and swelling of both upper and lower lips with angular cheilitis were noted (Fig. 1(B)). Punch biopsies from lip and vulval swelling were taken.

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Figure 1.

(A) Diffuse vulval swelling presenting as indurated firm swelling of both labia majora and minora, (B) swelling of both upper and lower lips with yellowish crusting at left angle of mouth, (C) HE X100: Biopsies from lip and vulva lined by stratified squamous epithelium with subepithelium showing multiple epithelioid cell granulomas with mild chronic inflammation, (D) HE X400: Non-caseating epithelioid cell granulomas with multinucleated giant cells. Reduction in vulval swelling (E) and lip swelling (F) on follow-up.

These revealed lining stratified squamous epithelium with subepithelial tissue showing non-caseating epithelioid cell granuloma with mild chronic inflammation (Fig. 1(C) and (D)) with absence of Acid-fast bacilli, Lepra bacilli, fungus, parasite or calcification.

A diagnosis of simultaneous vulval and lip involvement in a known case of Crohn's disease was made and the patient was started on Metronidazole 400 mg bd and intralesional injection of Triamcinolone acetonide once weekly. Reduction in size of the swellings was seen on follow-up (Fig. 1(E) and (F)).

Discussion

Crohn's disease is a chronic granulomatous gastrointestinal disorder of unknown aetiology. The ileocecal junction is the most common site affected; however, it can involve any part of gastro-intestinal tract. Gastro-intestinal symptoms include recurrent aphthoid ulcers, abdominal pain, bloody or watery diarrhoea, together with the presence of systemic symptoms such as fever and weight loss. Clinically observed extra-intestinal manifestations include arthritis, scleritis, episcleritis and cutaneous manifestations such as perianal and peristomal lesions, pyoderma gangrenosum, Sweet's syndrome, acrodermatitis enteropathica, epidermolysis, bullosa acquisita and granulomatous cutaneous lesions. ¹

Oral manifestations may also occur in between 0.5% and 37%. They can be classified as specific lesions, such as diffuse lip and buccal swelling and cobblestones, and non-specific lesions, such as aphthous ulcers, pyostomatitis vegetans, caries, gingivitis and periodontitis. ²

Vulval Crohn's disease as a clinical manifestation is extremely rare with approximately 130 cases reported in the literature worldwide. It presents in two ways, either as a direct extension of intestinal involvement, such as fistulas or fissures, or as ‘metastatic’ Crohn's disease. ³

The median time to diagnosis of vulvar Crohn's disease is 3.5 years, as patients often see multiple specialists before a diagnosis is established. Although most patients (50–80%) with this condition have underlying gastro-intestinal Crohn disease, not all patients with vulvar Crohn's disease will go on to develop bowel disease. ⁴

Histopathology is helpful in diagnosis, revealing non-caseating epithelioid cell granulomas. However, it needs to be differentiated from other granulomatous conditions such as genital tuberculosis, cutaneous sarcoidosis, hidradenitis suppurativa, lymphogranuloma venereum, donovanosis, leprosy, granuloma annulare, foreign body reaction.^1,3

An extensive literature search has shown cases of isolated vulval and lip swelling as extra-intestinal manifestations of Crohn's disease, which may precede or coincide with gastro-intestinal manifestations. ¹ However, only one case of combined vulval and lip involvement of Crohn's disease has been reported before. ⁵

Diagnostic approaches include colonoscopy, sigmoidoscopy or proctoscopy and imaging modalities to explore gastro-intestinal involvement. The primary management of vulvo-vaginal and oral disease consists of combined pharmacological therapy of antibiotics, anti-inflammatory agents, corticosteroids, and immunosuppressants in conjunction with non-pharmacological and surgical interventions. Metronidazole monotherapy or in combination with steroids are effective in the management of vulval Crohn's disease with a high success rate. ¹