Abstract

Abstract Introduction/Objective Ectopic sebaceous glands with or without hair follicles are well documented in a variety of sites including the vulva, cervix, esophagus, and male genitalia. Ectopic sebaceous glands in the vagina, however, are extremely rare, with only 2 case reports and a third case described in a journal correspondence letter in the English literature. Methods/Case Report We present a 39-year-old patient with Laurence-Moon-Biedl syndrome with pseudotumor cerebri, retinitis pigmentosa/macular degeneration, chronic kidney disease status post renal transplant, obesity, diabetes insipidus, and a urethrovaginal fistula and hypoplastic vagina status post repair and vaginoplasty at age 3 months. High grade squamous intraepithelial lesion on a cervicovaginal Pap test prompted colposcopy, during which a confluent plaque non-staining with Lugol’s solution was noted on the right vaginal sidewall extending from the introitus to 4 cm distal to the cervix. Biopsy of this area revealed hyper- and parakeratosis along with sebaceous glands and hair follicles in the subepithelial stroma. Two theories are proposed in the literature regarding the origin of vaginal ectopic sebaceous glands/cutaneous adnexal structures. The first is a developmental anomaly with aberrant differentiation in-utero, as the lower two thirds of the vagina is normally endodermal in origin and the upper one third is derived from mesoderm, while cutaneous adnexal structures are ectodermal in origin. The second postulates sebaceous glands in the vagina arise from a metaplastic process after prolonged irritation or inflammation. We add a third possibility in this patient, that vaginal plastic surgery performed at 3 months of age involved tissue re- arrangement or grafting which introduced ectodermal structures to the vagina. The patient’s vaginal surgical records were not available for review. Results (if a Case Study enter NA) NA. Conclusion We add a fourth case of ectopic cutaneous adnexal structures in the vagina in a patient with Laurence-Moon-Biedl syndrome to the literature. The origin of these structures may have resulted from vaginal surgery as an infant rather than from a developmental anomaly or metaplasia.

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