Granulomatous Vulvitis: A Rare Case of Chronic Inflammatory Hypertrophy of Vulvar Labia Related to Ulcerative Colitis

Abstract

Granulomatous vulvitis (GV) is an idiopathic entity typically presenting with chronic, painless swelling of the genitals with histologic evidence of granulomatous inflammation. Granulomatous vulvitis can typically start as an acute inflammatory condition, which gradually transforms into a chronic disease with a relapsing and remitting course leading to swollen, indurated, and distorted external genitalia. Association of GV with Crohn’s disease is being increasingly recognized. However, the association of GV with ulcerative colitis is unreported. Here, we report a rare case of GV in a middle-aged Indian female with characteristic gastrointestinal involvement suggestive of ulcerative colitis. We hope to contribute to an earlier recognition and a better management of the vulvar and gastrointestinal lesions of ulcerative colitis.

Introduction

Granulomatous vulvitis (GV) is a rare idiopathic inflammatory condition, typically presenting with chronic, painless swelling of the genitals with histologic evidence of granulomatous inflammation. GV is an idiopathic entity but it has been observed in association with other granulomatous conditions such as granulomatous cheilitis, tuberculosis, sarcoidosis, and Melkersson-Rosenthal syndrome.[1,8] Association of GV with Crohn’s disease is being increasingly recognized.[1] However, the association of GV with ulcerative colitis is unreported. Here, we report the first case of GV in a middle-aged Indian female with characteristic gastrointestinal involvement suggestive of inflammatory bowel disease (ulcerative colitis).

Case Report

A 48-years-old female, known diabetic since last ten years, presented to dermatology outpatient department with complaints of painless swelling of genitalia since last 1.5 years and an ulcer in the left groin fold since eight months. On further inquiry, she complained of on and off abdominal pain, recurrent diarrhea, blood in stools, and gradual weight loss since last three years. No history suggestive of genital ulcer, sexually transmitted disease, joint pain, chest pain, shortness of breath, and eye complaints. Examination of vulva showed enlargement along with irregular thickening with multiple skin coloured to erythematous papules and nodules present over mons pubis and labia majora along with knife-cut ulcers present on bilateral groin [Figure 1a-c]. There was no lymphadenopathy. The presence of copious, thin, white, foul-smelling vaginal discharge was noted. On per speculum examination,, cervix was healthy, and vaginal walls were coated with homogenous white discharge. KOH and wet mount examination were normal, Gram stain showed Gram-positive bacilli >30/hpf along with multiple neutrophils, and culture showed acinetobacter sensitive to cefixime, ceftriaxone, and doxycycline for which she was given tablet doxycycline 100 mg twice a day for 14 days leading to resolution of discharge with clean multiple knife-cut ulcers over the left inguinal fold. Gastroenterologist’s opinion was sought for patient’s abdominal complaints. Provisional differential diagnosis of GV, tuberculous vulvitis, sarcoidosis, filariasis, lymphogranuloma venereum, and syphilis were kept.

Figure 1.

(a) Enlargement along with irregular thickening of vulva comprising of multiple skin colored to erythematous papules and nodules present over mons pubis and labia majora; (b and c) multiple knife-cut ulcers in the bilateral groin folds

Hematological investigations showed microcytic hypochromic anemia (7.0 g/dL). Angiotensin-converting enzyme levels, chest and abdomen X-rays, ultrasound abdomen, and Mantoux test were within the normal limit. Sexually transmitted infection evaluation, including VDRL test and serology for herpes simplex virus, hepatitis B, HIV, and chlamydia, was nonreactive. Faecal calprotectin (264 µg/g) and CRP (18 mg/dL) were raised. On colonoscopy, there was a loss of vascular pattern, and multiple pseudopolyps and deep ulcers with mild mucosal bleed were noted. Ulcerative colitis endoscopic index of severity (UCEIS) was nine, which indicated severe ulcerative colitis. Microphotograph from colonoscopic biopsy showed ulcerated colonic epithelial lining covered by acute inflammatory exudate [Figure 2a] and effaced architecture with focal areas of necrosis and giant cell formation along with granulation tissue formation [Figure 2b].

Figure 2.

(a) (H and E, 100×): Microphotograph from colonoscopic biopsy showed ulcerated colonic epithelial lining covered by acute inflammatory exudate; (b) (H and E, 100×): Focal areas of granulation tissue formation and effaced architecture with focal areas of necrosis and giant cell formation along with granulation tissue formation

Skin biopsies from nodule over mons pubis and ulcer edge were taken, which on hematoxylin and eosin staining shows necrotizing dense granulomatous inflammation with giant cells and focal ulceration [Figure 3]. The microscopic examination using special histochemical stains did not show Donovan body and acid-fast bacilli and blood smear failed to show microfilaria.

Figure 3.

(a) (H and E, 10×) and (b) (H and E, 40×): Hematoxylin and eosin staining shows necrotizing dense granulomatous inflammation with giant cells and focal ulceration

Based on gastrointestinal tract complaints, colonoscopic biopsy, and skin biopsy findings of granulomatous infiltrate, final diagnosis of GV with ulcerative colitis was made. Hence, the patient was given a short course of tablet prednisolone 40 mg along with tablet 5-aminosalicylic acid 1.2 g thrice a day for six weeks. After that, patient reported improvement with decreased frequency of diarrhoea and abdominal pain and the knife-cut ulcer also got healed. There was 25% improvement in vulvar swelling and induration. In view of patient’s diabetic status, corticosteroids were stopped and rest was continued. After three months, there was no gastrointestinal complaint, but knife-cut ulcers had recurred. She was started on tablet minocycline 100 mg in the morning and 65 mg in the evening along with tablet methotrexate 12.5 mg once a week for her cutaneous and gastrointestinal disease. Six weeks later, follow-up of the patient showed healing of ulcer and further reduction in the vulvar swelling [Figure 4].

Figure 4.

(a and b) The patient showed healing of ulcer and further reduction in the vulvar swelling after starting treatment

Discussion

Inflammation of the vulva can present as a manifestation of a localized problem or as part of a systemic disorder. GV presents as painless edema and persistent or recurrent erythema, usually affecting the genital area.[2] GV typically starts as an acute inflammatory condition, which gradually transforms into a chronic disease with a relapsing and remitting course leading to swollen, indurated, and distorted external genitalia. In few cases, lymphangiomas can develop over vulva and adjacent skin.

The etiology of GV is mostly unknown, and the histopathological features show similarity with Crohn’s and granulomatous cheilitis.[3,4] Role of infection by virus, and bacteria of low pathogenicity, hypersensitivity to foreign substances, and atopy have been proposed.[8] Nonspecific activation of cell mediated immunity results in local tissue damage caused by cytokines released from activated T cells and macrophages; however, specific subtype of giant cell infiltration in vulva has also been identified by a pathologist.[8] Ulcerative colitis is a chronic idiopathic inflammatory disorder involving colonic mucosa in a contiguous manner, commonly involving rectum and may extend to other parts of colon. However, involvement of anogenital area in the form of fissures, fistulas, ulcers, and abscesses are uncommon in ulcerative colitis but are reported more commonly in Crohn’s disease.[5] Patients of Crohn’s disease can present with variable vulvar symptoms, most common being vulval ulcerations (knife-cut ulcers or punctate ulcers). In few of the cases, labial swelling, exophytic growth, condylomatous growth, and pap smear abnormalities can be seen. [6,7]

GV itself is a rare condition that often presents both a diagnostic and therapeutic challenge. Moreover, the clinical presentation is varied and quite non-specific. Treatment options for this entity include immunomodulatory and anti-inflammatory drugs such as corticosteroids, hydroxychloroquine, systemic antibiotics, and surgical excision apart from symptomatic treatment.[8] The prognosis of GV is not known; however, because of chronic complications such as fibrosis and chronic lymphoedema, the vulva would be expected to remain swollen and distorted for long duration.[4] Persistent swelling can cause cosmetic and functional disability. Single case report of development of Bowen’s disease and multiple cases of adenocarcinoma in the vulvovaginal area affected by Crohn’s disease have been reported.[9] Since this is mostly associated with a systemic disease, thorough evaluation of the patient of vulvar or perianal erythema, edema, and ulceration with or without gastrointestinal manifestations is mandatory by a dermatologist. Detailed gastroenterological evaluation must be sought by the treating dermatologist for patients with esthiomene.

Conclusion

This is a rare case presenting as granulomatous vulvitis in association with ulcerative colitis. Cutaneous clinical presentation and prompt systemic examination and investigations lead to diagnose a long-standing inflammatory bowel disease in our patient and helped in timely management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.