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 .
 vulval involvement in crohn s disease ( cd ) is rare , particularly in children . 
 the clinical features include erythema , edema , ulceration , and labial skin tags . 
 the authors present two cases of children with vulval cd . in both cases , 
 marked labial edema was the presenting feature . 
 in one patient the immunomodulator tacrolimus ointment 0.03% was used with success . in the second patient control 
 was achieved with intralesional triamcinolone in combination with systemic metronidazole . 
Introduction
Methods
Case Series
Patient 1
Patient 2
Discussion
Conclusion