Metastatic vulvar Crohn′s disease-A rare case report and short review of literature

Abstract

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.

Crohn's disease of the vulva: A tough diagnosis (a case report of a 47 y.o. patient)

“…Thus, the prevalence of isolated vulvar CD may be higher than described but rarely diagnosed. The variability of the symptoms and characteristics of the course of vulvar CD also plays part in a considerable delay in the diagnosis verification . Cutaneous signs of vulvar CD normally include perineal edema and erythema accompanied by itching and/or pain, which, with further progression of the disease, are transformed into unilateral vulvar hypertrophy, abscesses, and ulcers with fistulas .…”

“…Changes in blood tests are nonspecific; the development of CD is usually not accompanied by any specific autoimmune markers. Vulvar CD can be diagnosed after ruling out all infectious and noninfectious diseases with similar clinical manifestations, such as actinomycosis, sarcoidosis, tuberculosis, candida infection, herpes virus infection, schistosomiasis, lymphogranuloma venereum, chronic lymphadenitis, cellulitis, hidradenitis, a foreign body, and neoplasia . Granulomatous inflammation without caseous necrosis is the main histological feature of CD, including its vulvar form .…”

“…The variability of the symptoms and characteristics of the course of vulvar CD also plays part in a considerable delay in the diagnosis verification. 3,6,9 Cutaneous signs of vulvar CD normally include perineal edema and erythema accompanied by itching and/or pain, which, with further progression of the disease, are transformed into unilateral vulvar hypertrophy, abscesses, and ulcers with fistulas. 3,8,10,11 Ulcerations in the inguinal folds resembling knife cuts are considered to be a pathognomonic symptom of vulvar CD.…”

“…Vulvar CD can be diagnosed after ruling out all infectious and noninfectious diseases with similar clinical manifestations, such as actinomycosis, sarcoidosis, tuberculosis, candida infection, herpes virus infection, schistosomiasis, lymphogranuloma venereum, chronic lymphadenitis, cellulitis, hidradenitis, a foreign body, and neoplasia. 9,10,12 Granulomatous inflammation without caseous necrosis is the main histological feature of CD, including its vulvar form. [1][2][3] This case report demonstrates that brush cytology of the damaged area surface was insufficient for morphological verification of vulvar CD.…”

Metastatic vulvar Crohn’s disease preceding intestinal manifestations: a case report and short review

“…The evolution of MCD can be unpredictable and sometimes refractory to medical treatment, although cases with spontaneous healing have also been reported (20). An established standard therapy for MCD remains underwhelming due to the lack of randomized control studies and definite guidelines (6,(20)(21).…”