The paraneoplastic pemphigus (PNP) is a neoplasia-associated autoimmune disease. It is characterized by a polymorphic mucocutaneous eruption, and its differential diagnoses are: pemphigus vulgaris, multiform erythema, Steven Johnson’s syndrome and lichen planus.
It was first described in 1990. PNP patients have reactive antibodies to desmoplakin and desmosomal plaque proteins present throughout their entire epithelium; thus, a more extensive involvement is not surprising.
The goal of the present report is to present a case of paraneoplastic pemphigus, which first manifestation were bullous lesions on the mucosae of the mouth, larynx and nasal septum.
A 68-year-old woman complaining of epistaxis and painful blisters in her mouth for thirty days, associated with dysphagia and weight loss – which she did not measure. She did not complain of skin lesions. She smoked and had blood hypertension, diabetes and dyslipidemia. Upon clinical exam she had ulcerated and bullous lesions on the palate, cheek mucosa and lower lip, and also an ulceration on her anterior nasal septum.
She also had ulcerated lesions with fibrin in her larynx and bilateral chorditis, seen upon telelaryngoscopy. Thinking about pemphigus vulgaris, she was started on prednisone, 40 mg/day and we biopsied the lesion on her cheek mucosa – which resulted inconclusive upon light microscopy and immunohistochemistry. After partial improvement only, the prednisone dose was increased to 60 mg/day.
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