Dysplastic Ichthyosis Uteri-like Changes of the Entire Endometrium

Ichthyosis uteri is an exceedingly rare condition in which the entire surface of the endometrium is replaced by stratified squamous epithelium. Originally described as an endometrial response to iatrogenically-introduced caustic substances, similar changes have since been described in association with a variety of inflammatory conditions of the endometrium. We describe herein a heretofore undescribed example of a moderately differentiated squamous cell carcinoma of the uterine cervix associated with extensive ichthyosis uteri-like changes of the entire adjacent endometrium. Additionally, the squamous epithelium of the latter also showed multifocal changes diagnostic of a low-grade squamous intraepithelial lesion. The potential genesis of this composite of findings is discussed, as is the neoplastic potential of ichthyosis uteri. It is concluded that a squamous cell carcinoma of the cervix extended proximally into the endometrium, and that there was a colonization of a pre-existing ichthyosis uteri by associated human papillomavirus. The possibility of significant cervical pathology should be considered when plaques of squamous epithelium with low grade dysplastic changes are identified in an endometrial biopsy or curettage. Clinical history A 38-year-old nulligravid female presented with complaints of a vague pelvic heaviness and a vaginal discharge. Her past medical history is significant for acquired immune deficiency syndrome (diagnosed 9 months previously) and Hepatitis A, B and C infections. Physical examination revealed a massively enlarged, barrel-shaped cervix with areas of ulceration on the ectocervix and a fungating mass protruding from the endocervical region. Following a biopsy, the patient underwent a type III radical hysterectomy, bilateral salpingo-oophorectomy and pelvic/paraaortic lymph node sampling. The procedure was well-tolerated and without any complications. She has had no evidence of tumor recurrence at last follow-up, 9 months after her surgery.