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 x 7 cm, hanging from the left labium minus [Figure 1]. She had minimal swelling of the left labium majus. There was a tiny ulcer near the superior commissure of the labia majora. 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. Her vagina, cervix, and uterus were normal. 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 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. Ziehl-Neelsen staining for Mycobacterium tuberculosis was negative [Figure 2]. Special stains for fungal organisms were not performed.
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.
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, a diagnosis of CCD was made.
The patient was managed with metronidazole 400 mg t.i.d. for 1 week and 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. Later, the patient was lost on follow-up.
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].