|Year : 2021 | Volume
| Issue : 1 | Page : 98-100
Knife cut fissures: A clue for vulval crohn's disease!!
ML Ranjitha, Kanathur Shilpa, Budamakuntla Leelavathy, DV Lakshmi
Department of Dermatology, BMCRI, Bengaluru, Karnataka, India
|Date of Submission||15-Oct-2019|
|Date of Decision||15-Apr-2020|
|Date of Acceptance||03-Jun-2020|
|Date of Web Publication||19-Feb-2021|
Department of Dermatology, BMCRI, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
Crohn's disease is an inflammatory bowel disorder with various extraintestinal manifestations. Vulva is rarely involved in Crohn's disease and occasionally may constitute the only sign of the disease. It is often unrecognized and can be confused with various disorders involving the vulva. Vulval Crohn's disease is often refractory to treatment. We describe a case of 30-year-old female presented with labial swelling, discharging sinuses and knife cut fissures and diagnosed as a case of vulval Crohn's without intestinal involvement, that was managed with oral steroids, sulfasalazine, and metronidazole.
Keywords: Crohn's disease, knife cut fissures, metastatic Crohn's disease, vulval Crohn's disease
|How to cite this article:|
Ranjitha M L, Shilpa K, Leelavathy B, Lakshmi D V. Knife cut fissures: A clue for vulval crohn's disease!!. Clin Dermatol Rev 2021;5:98-100
| Introduction|| |
Crohn's disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. Cutaneous manifestations of Crohn's disease are among the most common extra-intestinal disorders and have been classified into specific or metastatic, nonspecific and reactive cutaneous manifestations. Gynecological involvement in these patients may include Bartholin gland abscesses, endovaginal fistulas, genital ulcers, or cutaneous Crohn's disease involving the vulvovagina, perineum, and labia. Vulval swelling, discharging sinuses, and fissures due to Crohn's disease is an extremely rare condition with only a few reported cases. We report a case of Crohn's disease of the vulva and perineum presenting as vulval swelling, discharging sinuses and knife cut fissures.
| Case Report|| |
A 30-year-old married woman presented with a history of vulval swelling and discharging skin lesions over perineum and perianal area of 2 years duration. She had a history of recurrent fever of 1 year duration. The past history revealed surgery done for hemorrhoids and vulval swelling 1½ year back but no medical records were available. The patient gave history of taking multiple oral medications with no improvement. No history of promiscuous behavior both in husband and wife. Family history was unremarkable. Menstrual cycle was regular and patient had two living children. History of pulmonary tuberculosis in father was present.
Clinical examination showed normal vital parameters and systemic examination. The patient had pallor. Local examination revealed few hyperpigmented firm pedunculated growths of size of about 3 cm × 4 cm with lobulated surface present over labia majora and minora [Figure 1]a and [Figure 1]b. Edematous, tender, hyperpigmented, thickening of skin was present over vulval, perianal, perineal and posterior aspect of both thighs. Two to three erythematous to hyperpigmented fleshy masses present over perianal area. Multiple, discrete, small, tender, sinuses with seropurulent, foul smelling discharge were present over perianal area and bilateral gluteal areas. The vagina and cervix were normal. Bilateral, nontender, inguinal lymphadenopathy was present. Our differential diagnosis at this stage was lymphogranuloma venereum (LGV), cutaneous tuberculosis, vulvoperineal Crohn's disease, actinomycosis, filariasis, deep fungal infections, and lymphangioma circumscriptum.
|Figure 1: Edema and hyperpigmented thickening of skin over vulva and both upper thighs with few lobulated swellings (a). Multiple discharging sinuses with hyperpigmented thickening of skin over perianal area (b)|
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The routine hematological investigation showed anemia, leukocytosis and raised ESR.
LFT, RFT, urine routine, HIV 1 and 2, VDRL, HBsAg, chest X-ray, and USG abdomen were normal. Fine-needle aspiration cytology of inguinal lymph node showed reactive lymphadenitis. Tissue smears for organisms and Donovan bodies were negative. Histopathological examination of a lesional skin stained with hematoxylin and eosin stain showed acanthotic epidermis, granulomas composed of epithelioid cells and multinucleated giant cells in the dermis and moderate inflammatory infiltrate composed of neutrophils, lymphocytes and few eosinophils [Figure 2]a and [Figure 2]b. Acid-fast bacteria, periodic acid-Schiff, and reticulin stain were negative. Mycobacterium and fungal culture, nucleic acid amplification test (NAAT) for mycobacterium were negative. In view of the clinical and histopathological features, a probable diagnosis of cutaneous tuberculosis was started on antitubercular drugs and patient started showing improvement.
|Figure 2: (a) Acanthotic epidermis granulomas composed of epithelioid cells and multinucleated giant cells. (b) In the dermis inflammatory infiltrate composed of neutrophils, lymphocytes, and few eosinophils (left, H and E, ×10)|
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However, again after 4 months of starting ATT patient developed multiple painful fissures in the groin, above the clitoris, interlabial creases, perineum, and perianal area [Figure 3]a and [Figure 3]b. Our diagnosis was revised and a diagnosis of cutaneous or metastatic Crohn's disease was made. The patient was referred further to gastroenterologist for further work up. Upper gastrointestinal endoscopy and colposcopy showed no gastrointestinal involvement was reported. The patient was started on oral prednisolone 30 mg/day which was gradually tapered, metronidazole 400 mg tid and sulfasalazine 500 mg bid. The patient started showing improvement within a period of 2 weeks [Figure 4]. All fissures healed within 4 weeks. The patient was subsequently lost for follow-up.
|Figure 3: Multiple painful fissures in the groin, above the clitoris, interlabial creases, perineum (a) and perianal area (b)|
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|Figure 4: (a) Healed lesions of clitoris and interlabial area. (b) Partially healed lesions in perianal area at day 12 posttreatment|
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| Discussion|| |
Crohn's disease, first described in 1922, is characterized by segmental granulomatous inflammation of the intestinal tract and frequently involves the cutaneous tissue as well. Cutaneous or metastatic Crohn's disease is a rare complication defined as the occurrence of specific granulomatous cutaneous lesions with the same histopathology (non caseating granulomas with multinucleated giant cells in the dermis surrounded by lymphocytes, plasma cells and eosinophils). Cutaneous Crohn's disease can occur in association with or preceding intestinal Crohn's disease. Vulvar Crohn's disease may initially present with swelling, erythema, pruritus or pain and subsequently develop unilateral vulvar hypertrophy, vulvar mass, vulvar edema, draining sinuses, ulceration, or abscesses. “Knife-cut fissures which resemble lacerations are almost pathognomonic of Crohn's disease although they have been reported in herpetic infections in the immunocompromised and in cutaneous tuberculosis.
Conditions which commonly pose a diagnostic dilemma for vulval Crohn's are cutaneous tuberculosis, LGV, hidradenitis suppurativa, filariasis, fungal infections. In our case, vulval Crohn's disease was initially misdiagnosed as cutaneous tuberculosis as it mimics clinically and histopathologically but Knife cut fissures and response to treatment helped in the diagnosis of cutaneous Crohn's. Cartridge-based NAAT test can help in differentiating cutaneous TB from Crohn's. Other infectious conditions such as actinomycosis, LGV, and fungal infections have to be ruled out by the demonstration of organisms or antibodies with biopsy, culture, or serological tests.
There are no definite guidelines for treatment of cutaneous Crohn's disease. Various treatments include intralesional and systemic corticosteroids, sulfasalazine, metronidazole, azathioprine, cyclosporine, methotrexate, thalidomide, infliximab, adalimumab, and surgical excision.
In our case, the patient responded well to oral steroids, sulfasalazine, and metronidazole.
| Conclusion|| |
Metastatic vulvar Crohn's disease poses a diagnostic dilemma for the treating physician because it may mimic many cutaneous conditions both clinically and histopathologically. Hence, vulvar Crohn's disease should be kept in mind while treating the patient with vulvar edema, swelling, draining sinuses, and knife cut fissures may serve as a clue for diagnosis and may prevent a delay in diagnosis and treatment of the condition.
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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]