Authors: Danielle Nicolle Dionisio Mejia, MD, Mae Ramirez-Quizon, MD, FPDS


Introduction: Pemphigus vulgaris is an autoimmune blistering disease predominantly seen in patients between 40-60 years with an incidence between 0.076 and 1.6 per 100,000-person years. Most cases of pemphigus vulgaris are seen in adults but also occur in adolescents in 1.4-3.7% of cases. The majority of pemphigus of childhood onset is of vulgaris type with onset at 12 years with no gender predilection and called juvenile pemphigus vulgaris. We present a 14-year-old female who presented with mucocutaneous lesions of pemphigus vulgaris with modest improvement on oral corticosteroids.

Case Summary: A 14-year-old female presented with one-month history of increasing number of painful oral ulcers treated with alum with no relief. Cutaneous examination revealed multiple erosions and fissures with hemorrhagic crusts on the lips, multiple discrete violaceous round plaques with indurated borders and flaccid bullae with hemorrhagic crusting on the flexor area of the right wrist, right forearm, and right elbow. Asboe-Hansen and Nikolsky sign were positive. Histopathology revealed a suprabasal blister with abundant dyskeratotic cells and direct immunofluorescence showed intercellular deposition of IgG diagnostic of pemphigus vulgaris. The patient was started on prednisolone at 1.5mg/kg/day. Modest improvement was seen after 1 week on prednisolone and complete resolution was achieved within a month of treatment.

Conclusion: Pemphigus vulgaris is a rare autoimmune blistering disease more commonly seen in adults. It may be rare in adolescents, it is important to consider this diagnosis and a raise a high index of suspicion in patients presenting with mucocutaneous erosions leading to a proper diagnosis and prompt treatment.





Pemphigus Vulgaris, Juvenile Pemphigus Vulgaris, Prednisolone

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