(Last Updated On: October 30, 2018)

Authors: Margaret Stephanie L. Jimenez MD, Karen Lee P. Alabado-Laurel MD FPDS

Abstract

Introduction: Pemphigus vulgaris (PV) is a chronic, autoimmune bullous disease with a potentially fatal outcome. It typically presents with flaccid blisters and erosions on both the skin and mucous membranes due to loss of coherence among keratinocytes. It is an uncommon disease with an incidence rate estimated at 0.5 to 3.2 per 100,000 per year. However, studies from Asian countries report that PV is the most common subtype of pemphigus with a male to female ratio estimated at 1:1 to 1:2. It generally affects patients aged 40-60 years old.

Case Summary: We present a 23-year-old male with a five month history of multiple flaccid bullae evolving into erosions with crusting on the face, oral mucosa, chest, back, and extremities. This was associated with weakness, odynophagia, weight loss and loss of appetite. Nikolsky sign was positive. Diagnosis was confirmed through skin biopsy revealing suprabasilar acantholysis consistent with an intraepidermal blistering disorder. He was treated with intravenous antibiotics and later started on systemic steroids leading to improvement of lesions. This was then shifted to oral steroids until azathioprine was introduced as a steroid sparer. However, patient would still report recurrence of lesions.

Conclusion: This is a case of PV presenting at an uncommon age with widespread cutaneous and oral mucosal affectation. It is associated with a high mortality rate due to its numerous comorbid symptoms and side effects of therapy. This report highlights the significance for early diagnosis and treatment as this determines the prognosis and clinical outcomes of the disease.

 

 

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Keywords

intraepithelial vesicle, pemphigus, erosions, desmogleins

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