Atypical Clinical Presentation of Pemphigus and Its Management: A Case Report

Introduction Many mucocutaneous conditions affect the skin and oral cavity. Common conditions include lichen planus, pemphigus, and pemphigoid. This case report shows an atypical presentation of a mucocutaneous condition wherein histopathology was required for definitive diagnosis and treatment. Case Presentation A 51‐year‐old African American male self‐reported to a private periodontal practice with a chief complaint of sporadic painful oral lesions of about 2 months duration. The patient also reported painful throat lesions. He had been previously treated for a sore throat followed by unspecified inflammation of the epiglottis. Clinical examination revealed diffuse bilateral eye redness, numerous intraoral ulcerations on soft and hard palate and floor of the mouth with trouble swallowing. Oral and ocular presentation suggested a differential diagnosis of cicatricial pemphigoid. Pemphigoid and lichen planus were also considered. Ophthalmologic exam revealed only allergies and no other abnormality. Soft tissue biopsies showed intraepithelial separation between the spinous layer and basement membrane. Direct and indirect immunofluorescence showed strong immunoglobulin G and moderate C3 reactivity confirming a diagnosis of pemphigus vulgaris (PV). A rheumatologist initially treated the patient with a daily combination regimen of methylprednisolone and mycophenolate mofetil. Lesions improved after a few weeks on the regimen. Reduction of medications resulted in return of oral and throat lesions. Resumption of the regimen resulted in lesion remission. Azathioprine replaced the mycophenolate mofetil, and then was gradually stopped after methylprednisolone cessation. Lesions have not reappeared for 1 year. Conclusion This case illustrates that early diagnosis of mucocutaneous lesions leads to proper treatment and possible remission.