Mucosa-predominant pemphigus vulgaris with anti-desmocollin 2 and 3 antibody positivity and ocular symptoms

A 24-year-old Japanese female presented to our department complaining of redness of eyes and pain in the mouth and throat. She had no particular medical history. She was treated in dentistry of our hospital, but there was no improvement. Stomatitis was exacerbated, and conjunctival hyperemia and hoarseness appeared, so she was referred to otolaryngology and ophthalmology of our hospital. At that time, she had hyperemia and blistering of the bulbar conjunctiva and pseudomembrane formation in the palpebral conjunctiva, and multiple ulcerative lesions of the oral cavity, pharynx and larynx (Fig.1a-d). Her symptom was suspected pemphigus vulgaris (PV), so she was referred to a dermatologist. Biopsy specimen from the lip revealed blisters and acantholytic cells in the epidermis with the infiltration of lymphocytes and neutrophils (Fig.1e). In the direct fluorescent antibody method, IgG and C3 are weak-positive deposited between epidermal cells (Fig.1f).