Ichthyosis Uteri

Author Information

Sathe Pragati A*, Patil Lalita Y**, Vaideeswar Pradeep***, Mayadeo Niranjan M****
(* Associate Professor, ** Assistant Professor,  ***Additional Professor, Department of Pathology, **** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India.)


Ichthyosis uteri, a rare condition, is called so when the entire surface of the endometrium is replaced by keratinized stratified squamous epithelium. It is a benign lesion and its association with benign and malignant conditions has been reported in the literature. If widespread keratinization of endometrial surface is detected in the curettage or biopsy then an advanced examination should be done to rule out an underlying malignancy.


The term ichthyosis uteri is used when there is widespread replacement of the surface endometrium by keratinized stratified squamous epithelium.[1] It is considered a benign lesion but its association with malignancy has been reported in the literature. [2] We report a case of ichthyosis uteri detected on endometrial curettage in an elderly postmenopausal woman.

Case Report

A 60-year-old woman, gravida 9, para 9 with eight living issues came with complaints of foul smelling white discharge of three months duration. Per vaginal examination showed a normal sized anteverted uterus. Bilateral fornices were free. On per speculum examination, foul smelling cervical discharge was noted. The vagina was normal. No growth was appreciated in the cervix. Ultrasonography of the pelvis showed 3x2x1.5 centimeters sized collection inside the uterine cavity. Both ovaries were normal. Clinical impression was endometritis with pyometra. The hematological and routine biochemical investigations were within normal range. The patient underwent cervical dilatation along with drainage of 50 ml of profuse greenish foul smelling purulent discharge from the uterine cavity. Papanicolau stain of the cervical discharge showed mature keratinized squamous epithelial cells on a background of neutrophils. Fractional endometrial curettage was performed after six weeks and the material was sent for histopathological examination. The microscopy showed prominent neutrophilic infiltrate with necrosis. Bathing in this exudates, were seen strips of keratinized squamous epithelium. The normal endometrial glands were covered by this epithelium (Figure 1a). There was low grade dysplasia of the squamous epithelium (Fig 1b). There was no evidence of associated endometrial malignancy. A diagnosis of ichthyosis uteri was given. The patient was administered oral antibiotics and discharged with an advice for a hysterectomy. She refused further treatment and was lost to follow-up, despite explanation of the associated risks.

Figure 1. Ichthyosis Uteri. 1a. Keratinized squamous epithelium with endometrial glands beneath. (Hematoxylin and Eosin, 100x); 1b. Low grade dysplasia seen in the squamous epithelium. (Hematoxylin and Eosin, 400x)


First coined by Zeller in 1885, the term ‘ichthyosis uteri’ means widespread keratinization of the endometrium. [3] It is rarer compared to squamous metaplasia. Chronic trauma, repair, irritation, inflammation, foreign material, and estrogenic effects have all been implicated.[3] The etiology of endometrial keratinization is not well understood. This rare pathology has been seen in association with benign conditions like tuberculous endometritis, puerperal endometritis, endometrial polyps, hyperplasias, squamous papilloma and with pyometra as a result of cervical stenosis.[1,3] Hence, when ichthyosis is seen on endometrial curettage, one has to look for associated benign pathologies especially tuberculosis, endometrial polyps and hyperplasia.
According to some investigators, ichthyosis lacks malignant potential.[1] However, dysplastic and anaplastic changes in the squamous epithelium have been reported, which may predispose to   the rare endometrial squamous carcinoma in postmenopausal women.[1,2]
The main differential diagnosis to be considered before making a diagnosis of pure ichthyosis is the extension of squamous carcinoma of the cervix into the endometrial cavity. The primary tumor in such a case would have an infiltrating rather than a polypoid morphology. The extension of well differentiated squamous carcinoma from the cervix can be distinguished from ichthyosis by detailed examination of the lower genital tract, by the presence of koilocytic changes and the presence of dysplastic changes in the squamous epithelium which favors a diagnosis of squamous carcinoma extension from cervix. [1,3]
In our case, the predisposing factor seems to be repeated episodes of pyometra which is suggested by strips of squamous epithelium embedded within abundant neutrophilic exudates as seen on endometrial curettage. The low grade dysplasia seen in the epithelium could suggest the likelihood of associated squamous carcinoma cervix. Unfortunately, our patient declined any further treatment and hence the cause of ichthyosis could not be ascertained
To conclude, ichthyosis is a benign condition but can lead to or be associated with endometrial malignancy, hence the need for timely recognition of this condition.[2] The clinical diagnosis in such cases is most frequently pyometra which has to be thoroughly investigated, especially when it occurs in postmenopausal women. If widespread keratinization of endometrial surface is detected in the curettage or biopsy then an advanced examination should be done to rule out an underlying malignancy.[1]


1.      Fadare O. Dysplastic Ichthyosis uteri-like changes of the entire endometrium associated with a squamous cell carcinoma of the uterine cervix. Diagn Pathol 2006;1:8-11.
2.      Bagga PK, Jaswal TS, Datta U, Mahajan NC. Primary endometrial squamous cell carcinoma with extensive squamous metaplasia and dysplasia. Indian J Pathol Microbiol 2008;51:267-8.
3.      Bewtra C, Xie QM, Hunter WJ, Jurgensen W.Ichthyosis uteri: a case report and review of literature. Arch Pathol Lab Med 2005;129:e124-e125.


Sathe PA, Patil LY, Vaideeswar P, Mayadeo NM. Ichthyosis Uteri. JPGO 2014 Volume 1 Number 8. Available from: http://www.jpgo.org/2014/08/ichthyosis-uteri.html