AI Article Synopsis

  • Uterine ichthyosis is a rare condition where the endometrial surface is replaced by keratinized squamous epithelium, which can pose risks of premalignant or malignant lesions.
  • A case study of a 66-year-old patient revealed high-grade dysplasia in the metaplastic squamous epithelium, highlighting the need for careful evaluation and potential surgical intervention.
  • The rarity of this condition calls for more research to create clear diagnostic and treatment guidelines, stressing the importance of clinical vigilance and teamwork in patient management.

Article Abstract

Uterine ichthyosis is a rare condition characterized by the extensive replacement of the endometrial surface with keratinized squamous epithelium. While generally benign, it can be associated with premalignant or malignant lesions, making its management critical. Here, we present a case of a 66-year-old patient diagnosed with uterine ichthyosis, highlighting the presence of high-grade dysplasia within the metaplastic squamous epithelium. This finding emphasizes the necessity for careful evaluation and appropriate surgical treatment to rule out potential associated neoplasms. Given the rarity of uterine ichthyosis and its implications for patient management, further research is warranted to establish clear guidelines for diagnosis and treatment. This case underscores the importance of ongoing clinical vigilance and interdisciplinary collaboration in managing patients with this condition.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11544502PMC
http://dx.doi.org/10.7759/cureus.71123DOI Listing

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Article Synopsis
  • Uterine ichthyosis is a rare condition where the endometrial surface is replaced by keratinized squamous epithelium, which can pose risks of premalignant or malignant lesions.
  • A case study of a 66-year-old patient revealed high-grade dysplasia in the metaplastic squamous epithelium, highlighting the need for careful evaluation and potential surgical intervention.
  • The rarity of this condition calls for more research to create clear diagnostic and treatment guidelines, stressing the importance of clinical vigilance and teamwork in patient management.
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Rationale: Ichthyosis uteri is a rare pathological condition characterized by the replacement of the endometrial lining by stratified squamous epithelium. Yet its occurrence with endometrial adenocarcinoma is very rare.

Patient Concerns: A 68-year-old woman has been experiencing sporadic, minor vaginal hemorrhages for a few months.

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Neu Laxova syndrome (NLS) is a rare and lethal congenital disorder characterized by severe intra-uterine growth retardation (IUGR), ichthyosis, abnormal facial features, limb abnormalities with arthrogryposis and a wide spectrum of severe malformations of the central nervous system (CNS). NLS is due to biallelic variants in three genes previously involved in serine-deficiency disorders (PHGDH, PSAT1 and PSPH), extending the phenotypic spectrum of these disorders.

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Background: Ichthyosis uteri is an extremely rare condition in which the entire or extensive parts of endometrial lining are replaced by stratified squamous epithelium. Malignant potential of this entity is unclear and its association with dysplastic changes and primary squamous cell carcinoma of the endometrium and endometrial adenocarcinoma has been reported. However, lack of data makes difficult to interpret the significance of neoplasms arising from this condition.

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