Chronic linear ulcerations of the inguino-crural and buttocks folds.

Vulvo-perineal Crohn's disease is a rare condition either when it is isolated or associated with digestive manifestations. In the former condition named metastatic Crohn's disease, it may constitute a diagnostic challenge and may be confused especially with other infectious or inflammatory disorders. We report a case of vulvo-perineal Crohn's disease in a 46-year-old woman. A 46-year-old woman was diagnosed with a vulvo-perineal Crohn's disease without digestive involvement. There was a chronic edema of the vulva with linear ulcerations on the inguino-crural regions and the buttocks fold, of 3 years. Treatment with metronidazole (1 g/day for 6 months) led to almost complete healing of the ulcerations with a sustained result. Physicians must be aware of the diverse manifestations and confusing presentations of vulvo-perineal Crohn's disease.

Case Report

A 46-year-old woman, having in her past medical history chronic juvenile arthritis with bilateral prosthetic hips, presented with painful vulvo-perineal ulcerations of 3 years. There was no diarrhea or recent weight loss. Cutaneous examination showed asymmetrical vulvar edema of the labia minora and labia majora with deep and linear ulcerations having verrucous borders located on the inguino-crural regions [Figure 1] and the buttocks fold [Figure 2]. On physical examination there was bilateral limited mobilization of the hips. A biopsy specimen was taken from the border of the vulvar ulceration. Histological examination showed under an epidermal hyperplasia [Figure 3], an epithelioid granuloma of the dermis showing multinucleated giant cells without caseation [Figure 4]. Direct immunofluorescence on cutaneous biopsy was negative. Biology and chest X-ray were normal. Koch's bacillus in sputum, microbiological studies (staining for micro-organisms and cultures) and tuberculin testing were negative.

Figure 1

Asymmetrical vulvar edema with deep and linear ulcerations of the inguino-crural regions

Figure 2

Linear ulcerations with verrucous borders of the buttocks fold

Figure 3

Epidermal hyperplasia (H&E stain, 100×)

Figure 4

Epithelioid granuloma of the dermis showing multinucleated giant cells without caseation (H&E stain, 400×)

Discussion

Our patient underwent colonoscopy and small bowel enterography showing no Crohn's disease aspect. Histological examination from intestinal biopsies is also with normal aspect. Based on the clinical (vulvar lymphedema with typical linear inguinal and buttocks fold ulcerations) and histological data (granulomatous histological aspect without caseation), the diagnosis of metastatic Crohn's disease without digestive involvement was obtained. The patient was started with metronidazole 1 g/day. After a 6-month treatment, there was an almost complete healing of ulcerations.

Vulvo-perineal involvement in Crohn's disease is rare and may constitute the sole manifestation of the disease since it may precede the bowel symptoms by several months to years in 20% of cases and may constitute a diagnostic challenge. Abscesses, draining sinuses, edema, and sharply demarcated ulceration of the perineum and/or vulva with usually verrucous borders (as demonstrated by our case) are the characteristic cutaneous manifestations of the disease. They are caused by direct extension from the involved bowel or by granulomas separated from the bowel by normal tissue. The latter form is usually named metastatic Crohn's disease. In the absence of digestive involvement, the diagnosis is based on clinical and histological aspects and on the absence of other clinical and paraclinical signs of a specific infection.

The differential diagnosis of metastatic Crohn's disease includes cutaneous tuberculosis, atypical mycobacteriosis, and pemphigus vulgaris especially the vegetated form of pemphigus vulgaris. These diagnoses are eliminated in our case. Indeed, microbiological studies, so as the direct immunofluorescence on cutaneous biopsy, were negative.

Because of the scarcity of metastatic Crohn's disease and in the absence of a randomized assay, treatment remains not standardized.[1] Multiple treatments have been tried. They include corticosteroids which can be administrated either topically or locally injected or orally ingested. They are often efficient with a frequent cortico-dependence and multiple side effects especially for oral steroids. Sulfasalazine may lead to a partial healing of cutaneous lesions, but is more efficient in digestive forms. Immunosuppressive agents such as azathioprine, methotrexate, and cyclosporine are also efficient but a long-term treatment may expose the patient to severe side effects. Immunosuppressive treatments should be indicated in digestive involvement and may be an ultimate option in resistant or severe forms of metastatic Crohn's disease.[2] Thalidomide also led to a satisfactory result in refractory vulvar ulcerations associated with Crohn's disease.[3] Recently, Prestonet al stated that infliximab is a good therapeutic option for recalcitrant vulvar Crohn's disease but no optimum regimen was provided.[4] Metronidazole seems to be a good therapeutic option since it has led in our patient and in several uncontrolled reports to complete healing of perineal lesions, with a persistent result.[25] Its efficiency is due to antibacterial, anti-inflammatory, and immunosuppressive actions.

Pelvic manifestations of Crohn's disease can be psychologically crippling. To optimize management, physicians must be aware of its diverse manifestations and confusing presentations.