Abnormal cervical smear in hereditary mucoepithelial dysplasia: a case report

Hereditary mucoepithelial dysplasia is a disorder that involves the mucosa, skin, hair, eyes, and lungs. It is an autosomal dominant condition, although sporadic cases have been reported. The syndrome is caused by an abnormality in desmosomes and gap junctions. We report a case of abnormal cervical smear in an affected individual. Vaginal, oral, and urinary cytological smears show a lack of epithelial cell maturation, large paranuclear cytoplasmic vacuoles, and cytoplasmic strand-shaped inclusions. Abnormal Pap cervical tests have been reported in affected individuals. It has been found that a misinterpretation of abnormal from a cervical smear may lead to an unnecessary hysterectomy. We believe this disorder should be brought to the attention of colposcopists because misinterpreted abnormal cervical Pap tests can lead to unnecessary treatment in such affected individuals.