A 23-year-old married female presented to our outpatient department with complaints of swelling of the vulva and ulcers on the perineal folds for 5 years. The swelling started in 2011 during 1st trimester of gestation, which was gradually progressive (1-5 cm in size). Few painless ulcers developed on the inner aspect of both thighs after 1 year, which gradually increased in number and size. Gradually, she developed pain in the perineum. There was no history of preceding fever, chronic cough, weight loss, oral ulcers, loose stool, and blood in stool. She gave a history of cesarean section, which was performed due to genital prolapse. Then, she was operated for prolapse, and later, biopsy was taken from the swelling to rule out postoperative lymphangioma. The biopsy showed nonspecific granulomas and chronic infiltrates. She was started on oral azathioprine, antibiotics, metronidazole, and steroids with some improvement in groin ulcers and a decrease in vulvar edema and induration. She was on and off treatment for past 3 years with partial relief at best. Meanwhile, she took antituberculosis treatment for 3 months from the gynecology department of a tertiary hospital but did not show any improvement.
The condition worsened, and the patient presented in our OPD with complaints of vulval swelling, fissuring, and typical "knife-cut" ulcers, as shown in Figure 1.
On examination, an irregular firm, lobulated swelling arising from the vulva was appreciated. There were multiple, oval to linear, typical knife cutting deep ulcers with well-demarcated margins, of size varying from 0.5 to 5 cm. The ulcers were tender and showed fresh bleeding.
We repeated a biopsy which showed epithelioid cell granuloma in the dermis with dense chronic inflammatory infiltrate comprising lymphocytes and plasma cells, as shown in Figure 2. Special stains for acid-fast bacillus and reticulin were noncontributory. Our differential diagnosis at this stage included vulvar CD, cutaneous tuberculosis, sarcoidosis, deep fungal infection, and lymphogranuloma venereum.
Colonoscopic biopsy was done from the terminal ileum, which showed only mild inflammatory changes without any features of granulomatous disease. Colonoscopy was normal. Anti-neutrophil cytoplasmic antibody (both p ANCA and c ANCA) and anti-saccharomyces cerevisiae antibodies were negative. Routine hematological investigations and angiotensin-converting enzyme levels were within the normal range. Hepatitis B surface antigen, enzyme-linked immunosorbent assay for HIV, and Venereal Disease Research Laboratory test were negative. Tissue fungal culture was negative. The chest X-ray was normal. Magnetic resonance imaging of the pelvis showed vulvar edema with soft-tissue thickening within superficial subcutaneous layer extending up to the perineum likely inflammatory. In view of the clinical and histopathological features and all other investigations, a diagnosis of CD of the external genitalia was made by the exclusion.
In view of the previous nonresponse, we planned to start biologic therapy in form of injection adalimumab 160 mg (8/8/16)-80 mg (22/8/16)-40 mg (6/9/16) s/c at an interval of 2 weeks for 1 year, along with azathioprine 50 mg bid.
There was significant healing of the ulcers after six cycles of adalimumab, as shown in Figure 3.