Geburtshilfe Frauenheilkd 2015; 75 - A43
DOI: 10.1055/s-0035-1551617

Ichthyosis uteri associated with high grade cervical intraepithelial neoplasia (CIN 3) and intrauterine device (IUD) – a rare cause of recurrent abnormal PAP smear

LC Horn 1, K Ried 2, N Dornhöfer 3, U Siebolts 4, AK Höhn 1
  • 1Institute of Pathology, Division of Breast, Gynecologic & Perinatal Pathology, University of Leipzig
  • 2Department of Obstetrics and Gynecology, Division of Gynecologic Cytology, University of Leipzig
  • 3Department of Obstetrics and Gynecology, Dysplastic Clinic, University of Leipzig
  • 4Institute of Pathology, Department of Molecular Pathology, University of Halle/Saale

Background: Ichthyosis uteri is an exceedingly rare condition in which the surface of the endometrium is complete of near-complete replaced by 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. Here we describe a case with associated CIN 3 and repeated incomplete resection by conisation.

Methods & Case Report: A 55-year old women presented with lost IUD. Within IUD-removal curreted material represented squamous epithelium with high-grade SIL. Colposcopy showed T2 transformation zone and major changes, confirmed by atypical PAP-smear (Pap IVa-p Munich Nomenclatur III) and colposcopic guided cervical biopsy with CIN 3. Consecutive konisation specimen represented CIN 3 with complete resection. But, endocervical and endometrial curetting showed isolated strings of CIN 3. Repeated PAP-smear after 6 weeks represented PAP Iva-p and PAP IVb-p. Within the re-konisation specimen CIN 3 was seen, again with complete resection. But, endocervical and corpus curetting represented strings of CIN 3. Five weeks after re-konisation a simple hysterectomy was performed. The endometrium represented atrophic change and an ichthyosis uteri with high grade squamous intraepithelial lesion with p16 block staining.

Comment: Ichthyosis uteri is a rare condition with about 50 reported cases. 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 (Fadare 2006, Kabukçuoğlu & Arik 2011). The majority of pure squamous cell carcinoma of the endometrium lack any association to HPV-infection and p16-alterations (Horn et al. 2006). But some cases occur in association with ichthyosis uteri based on CIN 3 or squamous cell cervical carcinoma (Fadare 2006, Takeuchi et al. 2012). So, careful sampling and examination is required.