Abstract:

Pemphigus Vulgaris (PV) is an autoimmune blistering disease that affects the skin and squamous mucosa, and involves the genital mucosa in about 50% of patients. We present a 51-year-old female, with no history of squamous intraepithelial lesion (SIL), whose recent Pap showed atypical squamous cells of undetermined significance (ASCUS) characterized by crowded sheets of immature squamous cells with overall reparative changes, but with high nuclear/cytoplasmic ratios and enlarged nuclei with very prominent and frequently rectangular-shaped nucleoli. Nuclear contours were mostly smooth, and chromatin was regular, fine and pale. Further investigation revealed a history of PV, with one prior episode of genital involvement. At the time of her gynecological examination, oral lesions were noted, but no visible lesions were seen on colposcopy. Acetic acid application identified two white lesions, and biopsy demonstrated suprabasal clefts with acantholysis, compatible with PV. No SIL or malignancy was seen. The cytologic nuclear and nucleolar features observed in this case are typical of the few reported cases of PV changes in Pap test slides. However, the changes are not diagnostic, and the cytologic differential diagnosis includes non-specific reactive/reparative changes and malignancy. SIL is not likely, as prominent nucleoli are not a feature of SIL. Provision of the history of PV helps avoid overdiagnosis of the Pap as malignant/suspicious, which, in turn, helps to avoid excessive investigative procedures. Colposcopy with biopsy should be sufficient to confirm PV and rule out malignancy.

Financial Disclosure:
No current or relevant financial relationships exist.

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Ziba Rahbar, MD, MPH