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Idiopathic chronic granulomatous vulvitis and related diagnostic and treatment conundrum
  1. Smrati Sabnani1,
  2. Amrita Gaurav1,
  3. Nishank Manohar2 and
  4. Shweta Azad3
  1. 1Obstetrics and Gynaecology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
  2. 2Burns and Plastic Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
  3. 3Pathology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
  1. Correspondence to Dr Amrita Gaurav; aamrity{at}


Three years ago, a woman in her late 30s presented with huge masses in the lower genital tract region, including bilateral labia majora, labia minora and the gluteal region. The patient had had many antimicrobial, antiprotozoal, steroid and topical antibiotic treatments. The patient was misdiagnosed and treated for metastatic Crohn’s disease, but there was no improvement. The patient underwent extensive local excision of the lesions and was identified with a deep aggressive angiomyxoma; however, the tumors recurred. Her clinical condition deteriorated, and she was brought to this institution. This area was biopsied, and the results were suggestive of non-necrotising granulomatous disease. Labial and gluteal lesions were surgically excised, followed by primary closure of the operative site. The patient has undergone regular monitoring and has not experienced a recurrence.

  • Obstetrics and gynaecology
  • Dermatology

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Chronic granulomatous vulvitis is an uncommon inflammatory condition characterised by asymptomatic anogenital enlargement. Lymphoedema and non-caseating granulomatous inflammation are the pathogenic characteristics. This entity may be related to Crohn’s disease, sarcoidosis, Mycobacterium tuberculosis and granulomatous cheilitis. Histological similarities to Crohn’s disease make diagnosis difficult. Therefore, a clear diagnosis is essential prior to proceeding with additional treatment. We report a case of chronic granulomatous vulvitis with no other clinical signs and a diagnosis and treatment delay of over 3 years. The purpose of this case report is to underline the importance of a proper diagnosis of chronic granulomatous vulvitis, as well as to identify and rule out potential differential diagnoses, with the ultimate goal of achieving optimal care.

Case presentation

A woman in her late 30s reported recurring significant swellings in the vaginal region including bilateral labia majora and minora as well as bilateral gluteal folds for 3 years. She initially noticed swelling in her right labia majora 5 years ago, when it measured approximately 2×2 cm. The swelling was mildly uncomfortable, itchy and accompanied with a pinpoint opening with watery discharge. Over the course of 2 months, the size of the swelling increased to around 5×5 cm, and the patient experienced difficulties walking. She did not experience any gastrointestinal, respiratory or menstrual issues. Normal bowel and urinary habits were observed. She visited a healthcare centre, where the lesion was biopsied and found to be suggestive of advanced Crohn’s disease. Antimicrobials (doxycycline 100 mg for 3 months) and steroids (prednisolone 30 mg/day for 6 months) were administered as part of her medical care, but the lesion continued to grow in size. She underwent extensive local excision of the lesion at a different tertiary hospital. Histopathology results suggested the presence of an angiomyxoma with positive margins. She did not undergo adjuvant treatment following resection. Thetumour reappeared within 6 months. She was prescribed gonadotropin-releasing hormone analogues, injection leuprolide 11.25 mg for 3 months and anti-oestrogenic therapy in the form of tamoxifen for 6 months following the recurrence. No clinical reaction was elicited, and the masses and widespread oedema continued. She also received antifilarial medication in the form of 100 mg of diethylcarbamazine daily for 3 weeks. Due to the ineffectiveness of the previously described treatment, the patient visited the present institution.

On inspection, the patient was of average build, alert and oriented. She had considerable pallor, facial and bilateral pedal oedema up to the middle of the tibia. The right labia majora was replaced by an 8×5 cm, non-tender lesion. The left labia was replaced by a comparable 5×4 cm lesion. The clitoris was likewise replaced by a 2×2 cm polypoidal lesion. The right gluteal region was filled by an 8×10 cm indurated mass, whereas the left gluteal region had a 4×5 cm mass (figure 1). On speculum inspection, the vagina was swollen and the cervix was enlarged. The anterior vaginal wall, extending to the urethra, was indurated on vaginal examination. The uterus was of average size, anteverted, mobile and non-tender, and both fornices were free. Rectal examination was normal.

Figure 1

Extremely enormous lobulated labial enlargement involving bilateral gluteal folds, with thickened and oedematous overlying skin.


The patient was evaluated in our institute’s obstetrics and gynaecology department. Due to the inflammation and drug-induced Cushing syndrome, C reactive protein and serum cortisol levels were raised. The immune profile was within the normal range with the exception of a positive rheumatoid arthritis factor. Upper and lower gastrointestinal endoscopy revealed normal parameters. A test for faecal calprotectin, which is indicative of metastatic Crohn’s disease, was performed, which was negative.

Contrast-enhanced MRI of the pelvis and perineum revealed an ill-defined heterogeneously enhancing exophytic lesion with T2 heterogeneously hyperintense and T1 isointense, measuring 7×4.4×8.5 cm and arising from the labial folds and vulva. The lesion was observed to extend to the urethral orifice with diffuse fat planes. External and internal anal sphincters were oedematous and displayed enhanced fat planes with the rectum and anal canal. Multiple punch biopsy samples were taken, and histology revealed epidermal-localised ulceration and acute inflammatory cell collection. Dermis and subcutaneous tissue had an oedematous stroma containing numerous vascular channels surrounded by a plasma cell cuff and few histiocytes. There were a few scattered tiny epithelioid granulomas observed. Nevertheless, neither caseous necrosis nor Langhans large cells were observed (figures 2 and 3).

Figure 2

H&E staining at low magnification (10×) displaying oedematous dermis and subcutaneous tissue with many vascular channels and a perivascular lymphocytic cuff.

Figure 3

H&E staining at high magnification (40×) reveals a tiny epithelioid cell granuloma with Langhans large cells.

Differential diagnosis

Chronic idiopathic granulomatous vulvitis can be caused by a variety of clinical disorders, including infectious, inflammatory and miscellaneous causes. Infectious vulvovaginitis may be caused by Gardnerella vaginalis, Candida albicans or parasites (Trichomonas vaginalis). The clinical presentation of lymphogranulomatosis, actinomycosis, donovanosis, tuberculosis, syphilitic chancre, herpetic vulvitis (Herpes simplex virus -2), condyloma acuminatum (Human papilloma virus), hidradenitis suppurativa and Bartholin’s abscess may also be comparable. Inflammatory causes such as Crohn’s disease, Bechet’s disease and sarcoidosis can manifest as metastatic lesions in the vulva with a similar clinical presentation. Other disorders that may resemble chronic idiopathic granulomatous vulvitis include pyoderma gangrenosum, chronic eczema, epidermoid cancer of the vulva, foreign body reaction, post-radiation alterations, aggressive angiomyoma of the vulva and lymphatic blockage.


Following comprehensive consultation, the patient was scheduled to undergo surgical removal of the masses. Gynaecologists, plastic and reconstructive surgeons and anaesthesiologists comprised the team. The labial and gluteal lesions were surgically removed along with the primary closure of the operative site (figures 4 and 5). The patient developed a surgical site infection on postoperative day 4, which was treated conservatively. After 2 weeks, the patient was discharged with a healthy wound.

Figure 4

Intraoperative removal of large vulval and gluteal tumours.

Figure 5

Primary closure of surgical site with in situ bilateral 16 F subcutaneous drain.

Outcome and follow-up

The patient was followed up on a regular basis. After 1 month, she was readmitted owing to a wound infection that was treated with antibiotics and daily antiseptic wound dressing. Local application of Trimovate ointment (containing clobetasone butyrate, oxytetracycline calcium and nystatin) was prescribed. After 1 week, the patient was discharged with a healthy wound save for mild local oedema. The patient has been frequently observed in an outpatient clinic for the past 6 months (figure 6). The patient is now taking azathioprine tablets.

Figure 6

Wound’s condition 3 months after surgery.


Granulomatous vulvitis is an uncommon condition that primarily affects young adults and is characterised by painless vaginal enlargement and oedema with recurrent relapsing erythema. It was originally identified in 1979.1 Literature contains few descriptions of solitary cases, and there are no standardised management methods for this condition. The surgical treatment of chronic granulomatous vulvitis has been documented in roughly 10 cases.2 In the presence of intestinal involvement, the diagnosis of Crohn’s disease of vulval origin is straightforward. However, in the absence of intestinal involvement, the diagnosis is complicated due to the existence of other comparable illnesses.3 Ghosh et al described a case of severe granulomatous vulvitis without gastrointestinal signs of Crohn’s disease that was treated conservatively with oral flucloxacillin and symptomatic therapy.4 Van de Scheur et al reported a case study documenting three patients with anogenital granulomatosis in 2003. On histology, these lesions exhibited non-necrotising granulomas, oedema, lymphangiectasia and perivascular lymphocytic infiltration. They were treated with a combination of immunosuppressants and anti-tumour necrosis factor antibodies.5

Granulomatous vulvitis is particularly tough to treat. There is a lack of evidence to inform current treatment in the Indian population. Topical and intralesional corticosteroids, clofazimine, hydroxychloroquine and antibiotics such as metronidazole and minocycline are available as treatment alternatives.6 In case of vulvar Crohn’s disease, oral 5-aminosalicylates (mesalamine or sulfasalazine) are administered alongside corticosteroids and immunosuppressants such as azathioprine or ciclosporin.7 Anti-tumour necrosis factor monoclonal antibodies, such as adalimumab and infliximab, have also been administered with encouraging outcomes.8 Surgical resection in the form of vulvectomy may be required in cases involving substantial functional difficulty and cosmetic deformity, or lesions unresponsive to medicinal treatment.9

Patient's perspective

My family and I were profoundly scarred by the illness and therapy failures. I once considered taking my own life. After surgery, however, I have experienced a great improvement in my quality of life, and I am really thankful to the specialists who helped me achieve this.

Learning points

  • Chronic granulomatous vulvitis is a rare condition that might be initially challenging to diagnose.

  • If not recognised and treated promptly, it can cause significant aesthetic deformity and damage the patient’s psychosexual life.

  • For timely diagnosis and management of the condition, the patient must be referred to a tertiary care facility with a multidisciplinary approach combining gynaecologists, gastroenterologists, dermatologists, pathologists and plastic surgeons.

Ethics statements

Patient consent for publication


The authors would like to acknowledge the Department of Pathology, All India Institute of Medical Sciences, Rishikesh, India, for their valuable contribution to the histopathological images.



  • Contributors SS, AG, NM and SA have been actively involved in this patient’s management and care. SS and AG have formulated the manuscript. NM and SA have proofread the manuscript and added valuable suggestions. All the authors have read and agreed to the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.