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Year : 2016  |  Volume : 61  |  Issue : 1  |  Page : 70-74
Metastatic vulvar Crohn's disease-A rare case report and short review of literature

Department of Dermatology, Silchar Medical College, Silchar, Assam, India

Date of Web Publication15-Jan-2016

Correspondence Address:
Debajit Das
Department of Dermatology, Silchar Medical College, Silchar, Assam - 788 014
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.174028

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Metastatic Crohn's disease (CD), a type of extraintestinal CD may present with gynecological manifestation which causes diagnostic dilemma and needs multidisciplinary approach. Vulvar lesions occur in very small number of cases with CD of which asymmetrical labial swelling and edema is the most common presentation. We report a case of hypertrophic exophytic variety of vulvar CD because of its rarity.

Keywords: Crohn′s disease, metastatic, vulvar

How to cite this article:
Das D, Gupta B, Saha M. Metastatic vulvar Crohn's disease-A rare case report and short review of literature. Indian J Dermatol 2016;61:70-4

How to cite this URL:
Das D, Gupta B, Saha M. Metastatic vulvar Crohn's disease-A rare case report and short review of literature. Indian J Dermatol [serial online] 2016 [cited 2022 Aug 10];61:70-4. Available from:

What was known?

  • Metastatic vulvar Crohn′s disease is a rare entity causing diagnostic difficulty to treating physician.
  • In 25% of cases it may precede the diagnosis of intestinal Crohn′s disease.
  • Asymmetrical labial swelling and edema is the most common presentation of vulvar metastatic Crohn′s disease.
  • Treatment is often difficult and needs a multidisciplinary approach and a combination of different therapeutic modalities.

   Introduction Top

Crohn's disease (CD) is a chronic relapsing and remitting inflammatory disorder of the gastrointestinal (GI) tract that can affect the entire length of GI tract from oral cavity to anus, characterized by abdominal pain, abdominal cramping, diarrhea and weight loss. [1] Extraintestinal manifestations of CD may affect up to 35% of patients because of its characteristic transmural inflammation and fistula formation. [2],[3] Metastatic CD is defined as having granulomatous lesions of CD in an area that is noncontiguous to the GI. [1] Vulvar lesions occur in approximately 2% of women with CD of which asymmetrical labial swelling and edema is the most common presentation. [3],[4] It may occur either as direct extension of the perineal region or due to metastatic disease. We here report a case of metastatic vulvar CD of hypertrophic exophytic variety because of its rarity.

   Case Report Top

A 30-year-old married woman reported to the Dermatology and STD OPD, in the month of August 2014, with complaints of a genital swelling since last 1 year. Swelling was progressive and initially started with small nodules of 2-3 cm diameter at the right labia majora. Within period of 6 months, it almost symmetrically involved both labia majora sparing the labia minora, clitoris and periurethral region. Gradually, within a period of 3 months, it also involved the perianal area. It was associated with mild itching. There was no history of premenstrual flare up of the lesions. For the last 2.5 years, she intermittently experienced low grade fever, malaise, abdominal pain and cramping and occasional bloody diarrhea for which she habituated to take over the counter medicine which reduced the symptoms sometimes and never took any consultation. The patient was housewife with a single child. She and her husband denied any premarital and extramarital exposure. There was no significant drug history.

On general examination, the patient appeared normal except for moderate pallor and poor nutrition. There was no lymphadenopathy or swelling of limbs.

Cutaneous examination of genital region revealed, both labia majora grossly thickened and hypertrophied and studded with multiple, almost bilateral symmetric nodules of different sizes varying from 0.5-5 cm diameter, some of which coalesced to form larger lesions. Localized areas with pus and blood-tinged pus were also seen. Thickened labia majora almost occluded view of labia minora. On a parting, normal looking labia minora, clitoris and periurethral region can be seen. There was no associated discharge per vagina. Per speculum examination did not reveal any abnormalities. Multiple nodules lying singly and coalesced to form a larger lesion were also seen in the perianal region [Figure 1].
Figure 1: Hypertrophic exophytic growth and studded with nodules, some of which contain pus and pus with hemorrhage

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Cutaneous examination of rest of body and systemic examination did not reveal any abnormalities.

Routine laboratory tests of blood, urine and stool were normal apart from Hemoglobin which was 8 gm%; total leukocyte count (TLC) 9200 cells/cubic mm; erythrocyte sedimentation rate (ESR) 40 mm at the end of first hour; Tridot screening for human immunodeficiency virus (HIV), VDRL and serological tests for other STDs including lymphogranuloma venereum were negative. The night blood for microfilaria was negative. Angiotensin-converting enzyme level for sarcoidosis was normal. Mantoux test and TB-interferon were negative.

Colonoscopy revealed aphthous ulceration in descending colon [Figure 2]. Tissue sample from the colon was inadequate and result came inconclusive. The patient refused a repeat biopsy.
Figure 2: Colonoscopy reveals aphthous ulceration in descending colon

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X-rays of chest and pelvic region were normal. CT scans of abdomen and pelvis were also normal.

Histopathology of lesion showed that the dermis was thickened and mildly fibrotic and had a moderately dense perivascular lymphocytic infiltrate and non-caseating granuloma of epitheliod cells. There was thickening of fibrous septa of the subcutaneous fat with small non-caseating epitheliod granulomas in the thickened fibrous septa [Figure 3], [Figure 4] and [Figure 5].
Figure 3: Dermis is thickened and mildly fibrotic and had a moderately dense perivascular lymphocytic infiltrate with non caseating epitheliod granuloma in the upper dermis. (H and E ×10)

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Figure 4: Non-caseating epitheliod granuloma with foreign body and Langhans type giant cells can be seen in the dermis ( H and E ×40)

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Figure 5: There is thickening of fibrous septa of the subcutaneous fat with small non-caseating granulomas in the thickened fibrous septa (H and E ×10)

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A final diagnosis of metastatic vulvar CD is made. The patient is put on Tab. prednisolone 40 mg/day along with Tab. metronidazole 400 mg three times daily. The patient was followed up for next 3 months and there was no significant improvement. After that patient did not come for follow up.

   Discussion Top

CD, also known as Crohn's syndrome and regional enteritis, is a type of inflammatory bowel disease (IBD) that may affect any part of the GI tract from oral cavity to anus. [1],[5] Cutaneous manifestations is not uncommon, may be classified as (i) peri-anal and peri-stomal lesion (the most common presentation); (ii) different skin lesions associated with CD: Erythema nodosum, pyoderma gangrenosum, Sweet's syndrome, acrodermatitis enteropathica, epidermolysis bullosa acquisita; (iii) granulomatous cutaneous lesions separated from the affected gut by healthy tissue known as metastatic lesions. [6] Over 50% patients of CD are women [7] and they may experience a variety of gynecological manifestations, including vulvovaginal involvement, which is often not easy to recognize and also difficult to treat. Among female patients with CD, 24% may have some form of gynecological association, which may occur before or after the confirmed diagnosis of CD. [8]

According to Feller et al., [3] gynecological complications of CD [Table 1] and its mechanism [Table 2] are as follows.
Table 1: Gynecological complications of Crohn's disease[3] [Feller et al.]

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Table 2: Mechanism of Gynecologic involvement of Crohn's disease[3] [Feller et al.]

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Metastatic vulvar CD, being a rare entity having very few cases reported in literature, causes diagnostic dilemma to treating physician. In 25% of cases it may precede the diagnosis of digestive CD [9],[10] that complicates the situation and often causes delay in diagnosis causing significant physical, mental and social morbidity to the patient and requires a multidisciplinary approach.

As per Barret et al., [6] clinically vulvar CD is of four main types:

1. Vulvar swelling or edema: Most common type, might affect both labia minora and majora or the vaginal wall, and is typically inflammatory and asymmetrical

2. Ulceration: Might be totally asymptomatic or tender, unique or multiple, aphthoid and superficial or deep with an indurated base. Linear "knife-like" ulcerations may extend to the groins whenever present are characteristic of genital CD

3. Hypertrophic lesions: Either extensive with the infiltration of a whole labia or localized and exophytic, then presenting as pseudo-marisca or pseudo-condyloma acuminata. Chronic inflammation and recurrent cellulitis over the period of time causes impaired lymphatic drainage resulting in lymphatic vessel destruction or obstruction

4. Chronic suppuration leading to abscess formation.

Main clinical presentation of vulvar Crohn's disease [Table 3] are as follows
Table 3: Main clinical presentation of vulval Crohn's disease[6][Barret et al.]

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Isolated granulomatous vulvitis, a distinct rare entity characterized by non-caseating epitheliod cell granulomas in dermis which may be a part of  Melkersson-Rosenthal syndrome More Details, which is a triad of granulomatous cheilitis, facial nerve paralysis and fissured tongue and sometime associated with CD. [11],[12],[13] Similar clinical and histological findings have been reported in males on the penis and the scrotum [14],[15],[16] suggesting a specific predilection of cutaneous CD for the genital region.

As the vulvar metastatic CD is a relatively rare entity and clinical lesions are nonspecific and cause diagnostic dilemma, histopathological analysis is a cornerstone of diagnosis along with clinical correlation. Epidermal ulceration, presence of subacute or chronic inflammatory infiltrate, together with non-caseating epitheliod granulomas strongly support the diagnosis. [6] In hypertrophic exophytic variety, epidermal ulceration not seen but features of lymphocytic obstruction as multiple dilated lymphatic vessels may be seen in the reticular dermis. [9],[17] Other causes of granulomatous diseases causing diagnostic confusion to be excluded by clincopathological correlation such as genital tuberculosis, sarcoidosis, hidradenitis suppurativa, lymphogranuloma venereum, syphilitic lesions. Other important differentials are fungal, bacterial, parasital infectious vulvovaginitis, donovanosis, herpetic vulvitis, Bechet's disease, pyoderma gangrenosum, condyloma, foreign body reaction, and vulvar neoplasia. [6] Appropriate biological tests are also very important to support or refute the diagnosis. When CD is suspected clinically or histopathologically, searching of digestive Crohn's is necessary. Proctoscopy, sigmoidoscopy, colonoscopy, upper GI endoscopy along with imaging like USG, CT scan and MRI of abdomen and pelvis can reveal the clues of this fistulating disease. [1],[3],[5],[6]

The evolution of vulvar CD is unpredictable, often refractory to medical treatment and requires surgical intervention although cases reporting of spontaneous healing are also present in literature. [18] Treatment of metastatic CD of the vulva has consisted of topical and systemic therapies. Before starting medical therapy, deep fistulas and pelvic abscesses need to be excluded by physical examination, proper internal pelvic examination, examination under anesthesia and appropriate imaging. [1] In the literature, treatment of vulvar CD has included corticosteroids, azathioprine, systemic sulfasalazine, cyclosporine, ciprofloxacin and long-term metronidazole. [1],[3],[4],[5],[6]

Corticosteroids are a cornerstone of the treatment of CD as the vast majority of patients also had luminal CD; use of systemic corticosteroids is widely reported. [1],[5],[6] Topically applied glucocorticoids are also beneficial for distal colitis and may serve as an adjunct in those who have rectal involvement plus more proximal disease. [5] Local administrations or injections of corticosteroids have not been followed by satisfactory results [10],[19],[20],[21],[22],[23] except in one case. [24]

Metronidazole is effective in active inflammatory, fistulous, and perianal CD and may prevent recurrence after ileal resection. [5] The most effective dose is 15-20 mg/kg per day in three divided doses; it is usually continued for several months. [5],[6] Ciprofloxacin, both for its antimicrobial and its nonspecific anti-inflammatory properties, may also be used. Both ciprofloxacin and metronidazole can be used in combination in active inflammatory, fistulizing and perianal CD. [5],[6]

Oral 5-aminosalicylates (mesalazine or sulfasalazine) also can be used, generally more effective in bowel disease rather than perianal disease [4] but treatment alone was effective as reported in few cases. [10],[20],[25]

Infliximab is a chimeric monoclonal immunoglobulin G1antibody against tumor necrosis factor-alpha and is often combined with an immunomodulator to treat moderate to severe and fistulizing CD. [1] Significant clinical improvement was also seen in vulvar CD when used alone [26],[27],[28] or when combined with azathioprine [1],[29] or methotrexate. [22]

Surgical intervention included partial or total vulvectomy, [30] laser vaporization [31] or excision of lesion. [11],[20],[32] It is needed of when lesions are refractory to medical treatment and caused significant aesthetic disfigurement such in hypertrophic exophytic variety. Surgical intervention become mandatory when associated with Bowen's carcinoma. [33]

   Conclusion Top

Metastatic vulvar CD may mimic many diseases both clinically and histopathologically and may cause dilemma and delay in diagnosis. Rarer presentation like hypertrophic exophytic variety of our case, the possibility of CD should be kept in mind, so that fast diagnosis can be made and benefit the patient at the earliest.

   References Top

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Van Assche G, Dignass A, Reinisch W, van der Woude CJ, Sturm A, DeVos M, et al. European Crohn's and Colitis Organisation (ECCO): The second European evidence-based Consensus on the diagnosis and management of Crohn'sdisease: Special situations. J Crohns Colitis 2010;4:63-101.  Back to cited text no. 2
Feller ER, Ribaubo S, Jackson ND. Gynecolgic aspects of Crohn'sdisease. Am Fam Physician 2001;64:1725-8.  Back to cited text no. 3
Amankwah Y, Haefner H. Vulvaredema. Dermatol Clin 2010;28:775-7.  Back to cited text no. 4
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Barret M, deParades V, Battistella M, Sokol H, Lemarchand N, Marteau P. Crohn'sdisease of the vulva. J Crohnscolitis 2014;8:563-7.  Back to cited text no. 6
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Andreani SM, Ratnasingham K, Dang HH, Gravante G, Giordano P. Crohn'sdisease of the vulva. Int J Surg 2010;8:2-5.  Back to cited text no. 9
Werlin SL, Esterly NB, Oechler H. Crohn'sdisease presenting as unilateral labial hypertrophy. J Am Acad Dermatol 1992;27:893-5.  Back to cited text no. 10
Guerrieri C, Ohlsson E, Ryden G, Westermark P. Vulv it is granulomatosa: Acryptogenic chronic inflammatory hypertrophy of vulvar labia related to cheilitis granulomatosa and Crohn's disease. Int J Gynecol Pathol 1995;14:352-9.  Back to cited text no. 11
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What is new?
Hypertrophic exophytic variety is a very rare presentation of metastatic vulvar Crohn′s disease.


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3]

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