Clinical Pathologic Correlation:

Clinical History: Fifty-one year old male of Indian descent with history of painful ulcers of 2 weeks duration involving the maxillary and mandibular gingivae. PMH: HTN, GERDS, treated for some time with Altace and Zantac. NKDA. Quit smoking 2 years ago. No lesions anywhere else. One week after the initial exam he was re-evaluated. The ulcers remained unchanged. A biopsy was performed and sent for routine histology and direct immunofluorescence (DIF)

Initial Differential Diagnosis for this patient: Acute allergic reaction, erosive lichen planus, vesiculo-bullous process (mainly pemphigus vulgaris), infection.

Final Diagnosis: Pemphigus Vulgaris (PV).

Review of Pemphigus Vulgaris

• Clinical: Multifocal painful oral ulcers are first manifestation in up to 75 percent of cases; almost invariably develop during the course of the illness. Other sites: pharynx, larynx and nasal mucosa - presenting symptoms: hoarseness, painful swallowing and bloody mucous discharge. 30-60 years of age. Positive Nikolsky sign (although not pathognomonic). This diagnosis is usually considered after lesions have been present
for weeks, months, or during the first relapse.
• Skin: Small, flaccid, fluid-filled bullae on normal appearing skin. Main sites: chest, back, scalp, face.
• Histopathology and Diagnosis: Suprabasal or intraepithelial clefting (clinical bullae). Tzanck cells: free floating/acantholytic cells within the cleft.
• DIF of perilesional tissue: intercellular deposition (basket-weave pattern) of mainly IgG (also IgM and C3) against adhesion molecules (desmoglein-3).
• Treatment and Prognosis: Oral PV responds well to systemic corticosteroids. Prognosis excellent. Mucocutaneous PV responds to combination of corticosteroids and other immunosuppressive agents. Prognosis good with adequate management. The disease may be fatal if untreated.
• Notes about DIF: Should be performed to diagnose/differentiate multiple, ulcerative or vesiculobullous processes. Biopsy should be obtained from non-ulcerated areas and placed in Michel’s transport medium. This allows examination of the epithelium, basement membrane and subepithelial connective tissue.

Michel’s medium is an unstable solution with a high concentration of ammonium sulfate. Its shelf life is limited and must be kept refrigerated. Unlike formalin, it is not a fixative, therefore cellular viability is lost if processing is delayed.

We thank Dr. Ofilio Morales for his kind support and for contributing with this case. The biopsy was read at OHN Laboratory.