cutaneous manifestations of crohn s disease ( cd ) typically present as lesions that are contiguous with the gastrointestinal tract , such as peri - oral and peri - anal lesions [ 1 , 2 ] . vulval involvement , mostly due to distant spread of granulomata ( metastatic cd ) , is rare , especially in children [ 2 , 3 ] . here , a 10-year - old girl presented to the dermatology department at york hospital with a 12-month history of asymptomatic erythema and swelling of the vulva . the patient was a healthy - looking girl with normal height and weight for her age . peau dorange  appearance , with a large skin tag in the anterior peri - anal area ( fig .  1 ) . examination of the oral mucosa , rectum , and the rest of the skin was unremarkable . the patient was treated with tacrolimus ointment 0.03% which resulted in good control of the disease . a year after the initial presentation 1vulval swelling noted in patient 1 vulval swelling noted in patient 1 a 13-year - old girl presented with a 9-month history of asymptomatic vulval swelling . the patient was diagnosed with cd at the age of 5  years and had been well - controlled on methotrexate . examination of the peri - anal skin showed fissures and skin tags ( fig .  2 ) . examination of the rest of the skin , oral mucosa , and other systems was unremarkable . biopsy of the affected vulval area showed typical features of non - caseating granulomata with negative stains and culture for organisms . the patient was treated with intralesional triamcinolone and oral metronidazole , which resulted in a clear improvement in terms of swelling after 3  months.fig . 2unilateral vulval swelling with erythema noted in patient 2 unilateral vulval swelling with erythema noted in patient 2 a 10-year - old girl presented to the dermatology department at york hospital with a 12-month history of asymptomatic erythema and swelling of the vulva . the patient was a healthy - looking girl with normal height and weight for her age . peau dorange  appearance , with a large skin tag in the anterior peri - anal area ( fig .  1 ) . examination of the oral mucosa , rectum , and the rest of the skin was unremarkable . the patient was treated with tacrolimus ointment 0.03% which resulted in good control of the disease . a year after the initial presentation a 13-year - old girl presented with a 9-month history of asymptomatic vulval swelling . the patient was diagnosed with cd at the age of 5  years and had been well - controlled on methotrexate . examination of the peri - anal skin showed fissures and skin tags ( fig .  2 ) . examination of the rest of the skin , oral mucosa , and other systems was unremarkable . biopsy of the affected vulval area showed typical features of non - caseating granulomata with negative stains and culture for organisms . the patient was treated with intralesional triamcinolone and oral metronidazole , which resulted in a clear improvement in terms of swelling after 3  months.fig . 2unilateral vulval swelling with erythema noted in patient 2 unilateral vulval swelling with erythema noted in patient 2 metastatic cd in children is rare , and most present prior to the diagnosis of cd [ 1 , 2 ] . parks et al . there have only been 16 cases of vulval metastatic cd in children reported in the literature . vulval cd may present as the first and only manifestation of cd , as seen in patient 1 , or after development of the disease , even if well - controlled otherwise , as in patient 2 . the clinical presentation of vulval cd is variable and may simply manifest as diffuse edema with infiltration or ulceration . the absence of gastrointestinal symptoms often makes diagnosis difficult in children ; however , the presence of peri - anal fissures and skin tags should raise suspicion for vulval cd . the differential diagnoses for vulval swelling should include sarcoidosis , infections ( e.g. tuberculosis , cellulitis , lymphogranuloma venereum , actinomycosis , pyogenic infections , hidradenitis suppurativa , intertrigo , syphilitic lesions ) , foreign body reactions , contact dermatitis , acquired lymphangiectasia , vascular malformations , and sexual abuse [ 46 ] . biopsy of the lesion is often necessary to reach a definitive diagnosis , revealing the typical appearance of a non - caseating granulomatous inflammation as seen in cd . as there is little correlation between the activity and/or severity of skin lesions and bowel disease , treatment of the underlying cd may not be effective against cutaneous cd , as was seen in patient 2 . currently , metronidazole alone and/or topical / oral steroids seem to be the most effective treatment for metastatic cd . other beneficial therapies include dapsone , tetracycline , azathioprine , 6-mercaptopurine , sulphasalazine , and oral zinc supplementation . surgical procedures , such as vulvectomy , simple excision , curettage , and debridement , are reserved for resistant cases . tacrolimus ointment is currently licensed for use as a second - line agent in the management of atopic eczema . in children , the licensed strength is 0.03% ; however , it is increasingly used in other inflammatory conditions , including a pediatric vulval case of pemphigoid . in conclusion , vulval swelling can be the first or subsequent manifestation of cd and dermatologists should have a high index of suspicion to facilitate early diagnosis and control of the disease .