Granulomatous vulvitis is a rare localized inflammatory disease characterized by a painless, swollen, indurated and
distorted vulva. Histopathologically, it shows chronic non-necrotizing granulomatous inflammation with edema,
fibrosis, and lymphangiectasia. Some cases are associated with granulomatous cheilitis or Crohn’s disease. So far,
only a few cases of idiopathic granulomatous vulvitis have been reported. A 43-year-old female presented with a
1-year-history of persistent swelling of the vulva. The lesion was enlarged, edematous, and indurated, and there were
clustered vesicles and papules on the labia major. Histopathologic examination of the swollen labia major showed
granulomatous inflammation composed of multinucleated giant cells and epithelioid cells, with numerous
lymphocytes in the dermis. Another biopsy specimen obtained from the clustered vesicles showed lymphangiectasia
with chronic inflammation. Based on these characteristic histopathologic features and clinical findings, the patient
was diagnosed with idiopathic granulomatous vulvitis. Herein, we reported a rare case of idiopathic granulomatous
vulvitis that developed in a healthy woman.
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