ABSTRACT
Introduction: Herpes simplex (HSV) and varicella zoster (VZV) viruses are harmful infectious agents in pregnancy due to their ability to impact maternal-fetal dyads through various modalities including vertical transmission, neonatal infection, and maternal morbidity. As a result, accurate diagnosis and prompt treatment of these infections in pregnancy is critical. Case: A 19-year-old primigravida presented to our tertiary care center at 30 weeks' gestation with vulvar swelling, burning, and pain. Workup included direct PCR testing of a particularly erythematous area of the vulva which returned positive for VZV. The patient was treated with a 10-day course of acyclovir with resolution of her symptoms. She later had a full-term spontaneous vaginal delivery outside of the infectious window with no significant morbidity for either her or her neonate. Conclusion: Although a rare presentation, the presence of a genital lesion or labial swelling during pregnancy warrants workup for VZV, particularly among patients known to be varicella nonimmune. If genital VZV is diagnosed during pregnancy, the development of contingency plans through interdisciplinary collaboration should be pursued to ensure a safe delivery and postpartum course for both the maternal-fetal dyad as well as other patients on the unit and the provider care team.
ABSTRACT
In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. EMPD presents as an erythematous plaque on apocrine gland bearing areas (i.e. vulva, perineum, perianal region, scrotum, and penis) in older men and women. It can be a focal, multifocal, or an ectopic process. Immunohistochemical staining allows for differentiation between primary and secondary EMPD in addition to the many other disease entities that clinically resemble this malignancy. When diagnosing a patient with EMPD, a full history and physical should be performed given the possibility of an underlying malignancy. Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease.