crohn 's disease , first described in 1922 , is characterized by segmental granulomatous inflammation of the intestinal tract that frequently involves the cutaneous tissues as well .
cutaneous crohn 's disease ( ccd ) is synonymous with metastatic crohn 's disease ( mcd ) .
a 20-year - old woman , mother of a 1.5-year - old child , presented with a swelling in the genitalia of 6 months duration .
cutaneous examination revealed a single , non - tender , pedunculated , polypoid growth , about 6  7 cm , hanging from the left labium minus [ figure 1 ] .
however , many scars were seen on the medial aspect of both the thighs , which were attributed to pruritic ulcers in childhood , details of which were unavailable .
systemic examination , including the eyes and oral mucosa , did not reveal any abnormality .
filariasis being endemic in cuddalore district of tamil nadu , elephantiasis of external genitalia due to filariasis , besides , lymphogranuloma venereum and granuloma inguinale were also considered .
( a ) single , non - tender , pedunculated , polypoid growth , about 6  7 cm , hanging from the left labium minus ( p ) and minimal swelling of the left labium majus , ( b ) multiple  knife - cut  ulcers on the external genitalia , in the inguino - crural fold , and in the interlabial creases a clinical diagnosis of soft fibroma was entertained and an excision biopsy of the nodule was undertaken .
histolopathological examintion ( hpe ) revealed multiple non - caseating granulomas , edema , and dense lymphocytic infiltration in the dermis .
multiple non - caseating granulomas in the dermis ( g ) ( a ) h and e , 20 ; ( b ) h and e , 40 a week later , at the time of suture removal , the patient presented with multiple typical  knife - cut 
ulcers on the external genitalia , in the inguino - crural fold , interlabial creases , and natal cleft , leaving no doubt in the diagnosis of ccd [ figure 3 ] .
biopsy from knife cut ulcers could not be carried out since the patient was not willing to undergo the procedure .
she did not have any intestinal symptoms or pain abdomen at any time in the past or present .
ulcers on the external genitalia in the inguino - crural fold , ( a ) interlabial creases , and ( b ) natal cleft on investigation , she was found to have hypochromic microcytic anemia ( hb : 10.5 gm / dl ) and mild neutrophilic leucocytosis .
other relevant investigations including mantoux test , ultrasonogram of the abdomen , and chest radiograph were not contributory .
both enzyme - linked immunoassay ( elisa ) test for hiv and vdrl test were negative .
the patient was referred to a gastroenterologist for further work up , and no gastrointestinal involvement was reported . with this background ,
ciprofloxacin 500 mg bid . for 10 days in addition to oral prednisolone at 30 mg / day , which was gradually tapered over a period of 6 weeks .
she reported after 2 weeks with ulcers showing signs of healing , and by 6 weeks , they resolved .
however , she reappeared 2 years later with massive swellings of both the labia majora , more marked on the left labium majus .
she gave a history of having had a full - term normal child , 3 months earlier , delivered by an elective lower segment caesarean section ( lscs ) in view of the edema of the labia majora [ figure 4 ] .
ccd is relatively rare with fewer than 100 cases reported in the literature . two - thirds of the patients were women with a mean age of onset at 34.5 years , though our patient had lesions much earlier at 20 years of age .
vulvar involvement in cd may be by virtue of contiguity as a direct extension of intestinal involvement or non - contiguous ( metastatic ) in which there is no connection between the vulva and the bowel . in a review by andreani et al .
, 25% of vulvar cd did not have any intestinal involvement at the time of vulvar lesion .
it is in these cases that making a correct diagnosis becomes difficult , as was the experience of the present authors , who also missed the correct clinical diagnosis initially .
mucocutaneous manifestations occur in up to 44% of patients , but they do not necessarily mirror intestinal involvement akin to our patient , who did not have any intestinal symptoms at any time .
no intestinal involvement was found in the present case , similar to that in other reports .
skin manifestations in cd can be categorized as either histologically specific or non - specific ( reactive ) .
specific lesions are characterized by non - caseating granulomas , similar to those seen in the intestinal lesions .
nonspecific or reactive skin manifestations include entities like pyoderma gangrenosum , erythema nodosum , and oral aphthae
. however , such non - specific manifestations were not observed in the present case . among patients with ccd ,
chronic edema leads to firm coalescing papules and fibrotic nodules , which could be mistaken for lymphatic obstruction .
tuberculosis and filariasis , more common conditions in this part of south india , were also considered initially , before the biopsy report was available .
the differential diagnosis of granulomatous vulvar lesions includes conditions like sarcoidosis , foreign body implantation , hidradenitis suppurativa , mycobacterial , or deep fungal infections and granuloma inguinale that were ruled out by the hpe and other relevant investigations .
treatment of cd requires systemic administration of various antibiotics , immunosuppressive agents , and anti - inflammatory agents such as corticosteroids , azathioprene , sulfasalazine , or 6-mercaptopurine .
infliximab and adalimumab , cyclosporine , and mycophenolate mofetil have been found beneficial by various authors in addition to surgical intervention for fistulae .
she was advised frequent reviews by the gastroenterologist to help in early identification of gastrointestinal involvement and complications and its management , but she was lost to follow - up ; however , she resurfaced 2 years later with another child of 3 months age and no specific complications except for the massive swelling of the labia majora .
 crohn 's disease , first described in 1922 , is characterized by segmental granulomatous inflammation of the intestinal tract and frequently involves the cutaneous tissues as well . 
 cutaneous crohn 's disease ( ccd ) is synonymous with metastatic crohn 's disease ( msd ) . 
 a case of ccd , without any gastrointestinal involvement is reported for its rarity . 