Ichthyosis Uterus – A Rare Premalignant Lesion of Endometrium

Ichthyosis (ikTHeosis) uterus is a rare benign metaplastic transformation of uterine endometrium into stratified squamous epithelium. The word ichthyosis (plural ichthyoses) comes from two ancient Greek (ixtus), meaning fish and disease. The metaplastic epithelium is parakeratotic with absent granular layer. Premalignant potential of this benign transformation has not been established due to the rarity of occurrence. We report a case of 72 year old female, post menopausal with the complaints of painless lower abdominal distension for past six months. She had no history of menorrhagia or dysmenorrhea in her previous menstrual cycles. USG abdomen was done. She was taken up for elective hysterectomy with a diagnosis of hydrometra. Total abdominal hysterectomy with bilateral salpingo-oophorectomy with pelvic lymph node sampling done. Peritoneal fluid cytology, peritoneal biopsy was done. Gross section of specimen was seen and the same sent for histopathological examination. Based on these histological findings a diagnosis of Ichthyosis uteri with focal High grade Squamous intraepithelial lesion of endometrium was made. In our case, there is a direct continuity of the squamous metaplasia of the endocervix and the endometrial lining. This supports the benign metaplastic spread of squamous epithelium from the transformation zone of the cervix into the uterine cavity. However, the stimulation of squamous metaplasia in uterine endometrium as a result of long term hydrometra is being hypothesised in our patient. The association of hydrometra, squamous metaplasia and dysplastic changes in endometrium are speculated.