Pemphigus Vulgaris with Extensive Mucocutaneous Involvement: Importance of Early Recognition of Oral Lesions – A Case Report.

Abstract

Background:
Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder usually presenting in 5th decade of life. The hallmark feature of PV is intraepidermal acantholysis which is mediated by autoantibodies against desmoglein 1 and 3. PV predominantly affects mucous membranes and skin. Many cases often present initially only with oral lesions after which cutaneous involvement is gradually noticed. Early diagnosis is critical from the point of view of managing these patients as delayed recognition can lead to significant morbidity and mortality due to complications. In patients of PV oral manifestations are frequently misdiagnosed initially, thereby resulting in delayed treatment. This report highlights the importance of early identification of oral lesions in cases of PV and underscores the need of multidisciplinary approach in these cases.
Case Report:
A 35-year-old female presented with 1 month history of painful oral ulcers. These ulcers initially appeared as fluid-filled blisters that later ruptured to form erosions. There was a history of similar lesions gradually developing over the chest and abdomen. Clinical examination revealed multiple oral erosions involving the buccal mucosa and palate along with flaccid bullae and erosions on the trunk. Nikolsky's sign was positive. Tzanck smear demonstrated acantholytic cells. Histopathological examination showed suprabasal clefting with a “row of tombstone” appearance of basal keratinocytes. Clinical features and histopathology confirmed the diagnosis of PV. The patient was treated with systemic prednisolone and azathioprine therapy along with topical therapy as well as supportive care.
Significant clinical improvement occurred within two weeks of starting therapy with reduced lesion formation and improved oral intake.
Conclusion:
This case underscores the importance of identifying oral manifestations of PV and doing further evaluation in selected cases. Early recognition and timely initiation of immunosuppressive therapy are important in preventing disease progression and improving outcomes. A high index of suspicion for possibility of PV in cases with oral ulcers is essential to reduce diagnostic delays.