Anogenital Crohn's disease with vitiligo.

Cutaneous Crohn's or anogenital granulomatosis is a rare disorder, which presents commonly as swelling and erosions on anogenital area and associated with features of intestinal Crohn's disease. We report a case of 23-year-old female who presented with vitiligo patches on back and legs for 1-year, painful edema and ulcers on anogenital area and oral cavity for 9 months and diarrhea with bleeding per annum for 1 month. Features on ultrasonography, multislice computed tomography scan, colonoscopy, and biopsy from nodule on anal wall were suggestive of granulomatous Crohn's disease. Thus, we present the case due to its rarity.

INTRODUCTION

Cutaneous Crohn's was first described by Parks et al., in 1965,[1] refers to extra intestinal mucocutaneous manifestations of Crohn's disease in which there are noncaeseating, granulomatous mucocutaneous lesions preceding or manifesting after systemic manifestations of intestinal Crohn's disease. The most common findings are edema and ulceration of anogenital area and oral cavity. Other dermatological conditions found to be associated with Crohn's disease include pyoderma gangrenosum, erythema nodosum, erythema multiforme, epidermolysis bullosa acquisita, polyarteritis nodosa and vitiligo.[234] Here, we report a case of anogenital Crohn's and its rare association with vitiligo.

CASE REPORT

The case we present here is about a 23-year-old female, presented with vitiligo patch on legs and back for 1 year and vulval swelling with anogenital and oral erosions for 9 months [Figure 1]. Examination of vulva showed erythema and edema along with whitish, foul smelling discharge. There were multiple, painful, nonhealing erosions seen on anogenital area and oral cavity [Figures 2 and 3]. She also had on- and off-history of loose stools for last 1 month. Investigations on admission had shown low hemoglobin 10 gm%, total count of 7100/cumm, differential count of N: 58, L: 34, M: 4, E: 4, B: 0, platelet count 4.89l acs/cumm. Erythrocyte sedimentation rate was 40 mm in 1st h, blood urea 10 mg/dL, serum creatinine 1 mg/dL, random blood sugar 80 mg/dL. Serum glutamic pyruvic transaminase 10 U/L, serum glutamate-oxaloacetate transaminase 54 U/L, serum bilirubin 0.8 mg/dl, prothrombin time 14 s, activated partial thromboplastin time 34 s, total protein 5.3 g, albumin: Globulin 2.9:2.4. Urine routine microscopy was normal. HIV, rapid plasma reagin, HBsAg, hepatitis C virus were negative. KOH smear was taken from vaginal discharge, which was negative and report of Gram-stain is not available. X-ray chest and abdomen was without any abnormality. Ultrasonography (USG) of patient showed 17 cm bowel segment with 15 mm thickened wall and narrowing of the lumen with the diagnosis of inflammatory bowel disease. Multislice computed tomography (CT) scan of the abdomen with pelvis showed mild circumferential wall thickening with stratification and increased enhancement. Findings were in favor of Crohn's with early sacroillitis. Colonoscopy showed dilated bowel segment with edema, erythema, telangiectasia, and few ulcers [Figure 4]. Biopsy from anal wall showed acute as well as chronic inflammation with noncaseating granulomas suggestive of Crohn's disease [Figure 5]. Patient was treated with tablet mesalamine (800 mg) eight hourly and capsule doxycycline (100 mg) and neomycin cream on ulcers. She went to a higher center for further surgical management and died of septicemia.

Figure 1

Clinical photograph of vitiligo patches on legs

Figure 2

Clinical photograph of patient showing vulval edema

Figure 3

Clinical photograph showing anal erosions

Figure 4

Colonoscopy showing erosions in the terminal ileum

Figure 5

Histopathological picture

DISCUSSION

Crohn's disease, first described by Sir Crohn et al. in 1932,[2] is a type of inflammatory bowel disease that may affect any part of the gastrointestinal tract from mouth to anus.[5] It affects 1 in 400,000-600,000 people globally with male: Female ratio of 1:1.2. The age of onset of cutaneous Crohn's is 10-69 years.[6] In our case, the female patient was in her 20's with features of Crohn's disease. The basic underlying etiology of Crohn's is currently unknown, though various theories suggest an interaction between environmental, immunological and bacteriological factors in genetically susceptible individuals (NOD2 gene).[27] The lesions in cutaneous Crohn's can be classified as: (1) specific lesions of cutaneous Crohn's disease, (2) reactive dermatoses, (3) cutaneous manifestations secondary to malabsorption, and (4) cutaneous manifestations secondary to treatment.[6] Cutaneous Crohn's disease can also be divided clinically into two forms: Genital (56%) and extra genital (44%). Approximately, two-third of children and half of adults with Crohn's disease present with genital involvement.[3] Anogenital signs and symptoms (in approximately 25% in illeal and 80% in colonic involvement) may appear before the other systemic manifestations and may be the first sign in the diagnosis of Crohn's disease. Cutaneous manifestations associated with Crohn's disease are erythema nodosum, pyoderma gangrenosum, epidermolysis bullosa acquisita, polyarteritis nodosa, and vitiligo.[389] In our case, vitiligo preceded the anogenital lesions. Association of Crohn's disease and vitiligo has been seen in other studies also.[91011] In the study by Tanusin et al.[9] incidence of vitiligo with Crohn's disease was observed in 10% of cases. McPoland and Moss have reported a case of Crohn's disease and vitiligo.[10] In both studies, vitiligo was concomitantly associated. The differentials of chronic vulval edema with erosions are granulomatous vulvitis, sarcoidosis, and chronic lymphedema due to obstruction, tuberculosis, subcutaneous mycoses, hidradenitis suppurativa and Langerhans cell histiocytosis.[11] Clinically, presence of chronic erosions, swelling, and erythematous discoloration of genitals with gastrointestinal symptoms and on investigation typical findings in USG, CT scan and colonoscopy favors diagnosis of Crohn's disease. Absence of lymphadenopathy, vegetating growth, scarring, and negative X-ray findings excludes other differential conditions. Although histological picture of cutaneous Crohn's may be indistinguishable from other granulomatous diseases, but histopathology of skin along with colonoscopy findings together leads to diagnosis of Crohn's disease. The treatment of anogenital Crohn's is usually palliative and symptomatic. Many treatment modalities are available as systemic steroids, sulfasalazine, mesalamine (active moiety of sulfasalazine), oral metronidazole, hyperbaric oxygen, and antitumor necrosis factor-α antibody (infliximab).[236] Surgery is required if intestinal involvement is severe. Our patient was treated symptomatically with tablet mesalamine (800 mg) eight hourly, capsule doxycycline (100 mg) and topically neomycin cream and was referred to higher center for operative procedures.