A 30-year-old married woman reported to the Dermatology and STD OPD, in the month of August 2014, with complaints of a genital swelling since last 1 year. Swelling was progressive and initially started with small nodules of 2-3 cm diameter at the right labia majora. Within period of 6 months, it almost symmetrically involved both labia majora sparing the labia minora, clitoris and periurethral region. Gradually, within a period of 3 months, it also involved the perianal area. It was associated with mild itching. There was no history of premenstrual flare up of the lesions. For the last 2.5 years, she intermittently experienced low grade fever, malaise, abdominal pain and cramping and occasional bloody diarrhea for which she habituated to take over the counter medicine which reduced the symptoms sometimes and never took any consultation. The patient was housewife with a single child. She and her husband denied any premarital and extramarital exposure. There was no significant drug history.
On general examination, the patient appeared normal except for moderate pallor and poor nutrition. There was no lymphadenopathy or swelling of limbs.
Cutaneous examination of genital region revealed, both labia majora grossly thickened and hypertrophied and studded with multiple, almost bilateral symmetric nodules of different sizes varying from 0.5-5 cm diameter, some of which coalesced to form larger lesions. Localized areas with pus and blood-tinged pus were also seen. Thickened labia majora almost occluded view of labia minora. On a parting, normal looking labia minora, clitoris and periurethral region can be seen. There was no associated discharge per vagina. Per speculum examination did not reveal any abnormalities. Multiple nodules lying singly and coalesced to form a larger lesion were also seen in the perianal region [Figure 1].
Cutaneous examination of rest of body and systemic examination did not reveal any abnormalities.
Routine laboratory tests of blood, urine and stool were normal apart from Hemoglobin which was 8 gm%; total leukocyte count (TLC) 9200 cells/cubic mm; erythrocyte sedimentation rate (ESR) 40 mm at the end of first hour; Tridot screening for human immunodeficiency virus (HIV), VDRL and serological tests for other STDs including lymphogranuloma venereum were negative. The night blood for microfilaria was negative. Angiotensin-converting enzyme level for sarcoidosis was normal. Mantoux test and TB-interferon were negative.
Colonoscopy revealed aphthous ulceration in descending colon [Figure 2]. Tissue sample from the colon was inadequate and result came inconclusive. The patient refused a repeat biopsy.
X-rays of chest and pelvic region were normal. CT scans of abdomen and pelvis were also normal.
Histopathology of lesion showed that the dermis was thickened and mildly fibrotic and had a moderately dense perivascular lymphocytic infiltrate and non-caseating granuloma of epitheliod cells. There was thickening of fibrous septa of the subcutaneous fat with small non-caseating epitheliod granulomas in the thickened fibrous septa [Figures 3-5].
A final diagnosis of metastatic vulvar CD is made. The patient is put on Tab. prednisolone 40 mg/day along with Tab. metronidazole 400 mg three times daily. The patient was followed up for next 3 months and there was no significant improvement. After that patient did not come for follow up.