Dermatology Online Journal

Perianal Crohn disease
Sioud Dhrif Asma, Youssef Soumaya, Jaber Kahena, Dhaoui Mohamed Raouf, Doss Nejib
Dermatology Online Journal 12 (7): 18

Department of dermatology, Military Hospital of Tunis, Tunisia. asma_sidh@yahoo.fr

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Abstract

Crohn disease is a chronic inflammatory disease characterized by sharply demarcated segments of gastrointestinal involvement from mouth to anus. Its perineal manifestations are among the most devastating and mutilating complications. They occur at any time and may precede the intestinal manifestations. Their most common presentations are perineal ulceration, fistula, and abscess. Proliferative and polypoid morphology of the cutaneous lesions mimicking warts and condyloma are rarely described. We report a 25-year-old woman with a 4-month history of confluent plaques of the perineal region with vegetant surfaces, suspected to be genital warts. The lesions progressed to fistulas, inducing deep ulcerations surrounded by pseudocondylomatous tumors. About 2 months prior to presentation she began to suffer from gastrointestinal symptoms and noted weight loss. Physical examination, endoscopic examination, and pathological interpretation led to the diagnosis of Crohn disease with perineal involvement being the initial presenting sign. Significant improvement was induced with prednisone (45 mg daily) and azathioprine. Our observation is notable for the pseudocondylomatous appearance and the dramatic response to medical treatment despite severe involvement. .

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The perineal manifestations of Crohn disease (CD) are among the most devastating and mutilating complications[1]. There is a great variation in the literature with regard to the frequency of perineal lesions in CD [2]. Perineal involvement can even precede the intestinal manifestations [3]. The proliferative and polypoid morphology of the cutaneous lesions, mimicking warts and condyloma, are rarely described [4].

We report a patient with perineal CD who had pseudocondylomatous vulvar and anal lesions preceding intestinal symptoms.

Clinical synopsis

A 25-year-old woman presented with a 4-month history of confluent plaques and nodules in the perineal region. Because there were verrucous, vegetant surfaces, genital warts were suspected. The patient received limited laser CO₂ vaporization. The lesions progressed to fistulas with large, deep ulcerations surrounded by pseudocondylomatous tumors. The patient also complained of a recent onset of gastrointestinal symptoms consisting of alternating diarrhea and constipation leading to a weight loss of 12 kg. These symptoms began 2 months after development of the perineal lesions. She had no risk factors for warts or condyloma acuminatum (no sexual partners and no history of warts).

Physical examination revealed multiple erythematous and skin-colored, tender papules and plaques with granulomatous vegetations and deep ulcerations involving the perivulvar, perineal, and perirectal region (Fig. 1). Proctologic examination showed fistulas of the anus on the left recto-vaginal septum and the left labia minora with discharge of pus from the vagina. Mild tenderness of the lower abdominal region was observed on palpation. There was no evidence of lymphadenopathy or any other abnormality.

Figure 1	Figure 2
Figure 1. Multiple pseudocondylomatous vegetations and deep ulcerations involving the perivulvar, perineal, and perirectal region Figure 2. Lymphohistiocytic infiltrate and noncaseating granuloma formation with giant cells containing elastic fibers (Hematoxylin and eosin X 20)

Laboratory investigations indicated hypochromic, microcytic anemia (hemoglobin 8 g/dl, mean blood cell volume 73 fl), leucocytosis (white blood cell count 17,530 /mm²), raised C-reactive protein and erythrocyte sedimentation rate, depletion of iron stores, hypoproteinemia, and hypolipemia. Bacteriologic culture of the fistula pus grew Esherichia coli. A chest X-ray was normal and the tuberculin skin test was not reactive. Colonoscopy showed edema of the ileocecal valve and multiple polypoid formations of the ascendant bowel without evidence of sinus tract or fistula formation. Multiple biopsies of the ileocecal valve, the bowel and perineal plaques were performed. The histopathology of colon specimens revealed chronic inflammatory infiltrate and granuloma formation. Histopathology of perineal specimens showed ulcerated fibromembranous tissue, diffuse chronic inflammation with a lymphohistiocytic infiltrate, and noncaseating granuloma formation with giant cells containing elastic fibers (Fig. 2). Periodic acid Schiff and Fite stain results were negative for both fungi and acid-fast bacilli. Tissue culture findings for bacteria, fungi and mycobacteria were negative.

The diagnosis of CD with initial perineal involvement was made. The patient was first placed on antibiotic therapy with cefotaxime (Claforan®) and metronidazole (Flagyl®). Then, she was treated with prednisone 45 mg/day with azathioprine (Immurel®). Her condition has improved dramatically after 2 months of treatment.

Discussion

Crohn disease is a chronic inflammatory bowel disease characterized by sharply demarcated segments of gastrointestinal involvement from the mouth to the anus. Typical histologic findings include ulcerations, crypts, abscesses, and infiltration of the lamina propria by lymphocytes. Noncaseating granulomas are found in 10-25 percent of specimens [5]. Associated mucocutaneous finding occur in 22-75 percent of patients [6].

Skin manifestations have been classified into three distinct categories [7]. The most common is perineal ulceration, fistula, or abscess attributed to contiguous extension of the pathologic process of CD in the gastrointestinal tract onto the adjacent skin. The second type is rare and characterized by granulomatous cutaneous lesions that are non-contiguous with the gastrointestinal tract (metastatic Crohn disease); these appear typically as ulcerations. Plaques, papules, and nodules have also been described [7].

The third type includes many conditions that have a strong association with CD, such as erythema nodosum, pyoderma gangrenosum, and epidermolysis bullosa acquisita.

Among mucocutaneous manifestations, contiguous perianal skin lesions are the most common findings; approximately 25 percent of CD patients present initially with perianal lesions [8]. They usually occur in the form of fistulas, granulomas, or abscesses. Several different classifications of perianal involvement in CD have been suggested. Tolia established a classification describing three degrees of severity of perianal CD [3]. The mild form includes anal fissure, anal fistulae, and perianal abscess. The moderate form includes granulomatous hypertrophic tissue, pseudo-Bartholin gland abscess, and multiple perianal fistulae and fissures with shallow ulcers. The severe form includes non-healing, gaping, raw areas with excavating ulcerations, loss of the perineal body, and metastatic CD involving the vulva and rectovaginal fistula. Our patient presented with a severe degree of perianal involvement; earlier diagnosis may have avoided this progression.

Gynecologic complications occur in about one third of patients and can precede active bowel manifestations. They usually appear as enteric fistulas to the vagina, uterus, ovaries, and vulva. Vulval hypertrophy and pseudocondylomata, as in our patient, are very rare and can appear with or without intestinal disease [4].

Perineal manifestations of CD should be considered in the differential diagnosis of many granulomatous conditions such as lymphogranuloma venereum, tuberculosis, atypical mycobacterial or deep fungal infections, sarcoidosis, Melkerson-Rosenthal syndrome, and foreign body reaction. Intestinal symptoms, endoscopic lesions, and granulomatous histology, and the negativity of special stains and cultures allow clinicians to make the diagnosis of perineal CD.

Numerous therapies for the treatment of perineal CD have been suggested; many cases of perineal lesions appear to be recalcitrant to treatment. First, good perianal hygiene is the crucial for managing fistulae and infection. Several reports suggest that metronidazole may be beneficial for perineal fistulae [9]. Immunosuppressive drugs including systemic corticosteroids have been used to treat perineal CD. Cyclosporine has been used to treat perirectal fistulae [10]. Moderate to severe disease is often resistant to medical treatment and proctocolectomy may eventually be required in the case of non-healing perineal CD. Despite the severe degree of the perianal involvement, our patient showed a dramatic response to medical treatment (metronidazole, cefotaxime, and immunosupressive therapy).

Conclusion

In patients presenting with perianal lesions without gastrointestinal symptoms, a high degree of suspicion is required and CD should be suspected. In fact, these lesions are often misdiagnosed as hemorrhoids or condyloma. Good results can be achieved by early, aggressive systemic therapy to avoid progressive local disease with severe mutilations and the necessity of surgical intervention.

References

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