LAUSR

An 89-year-old Chinese gentleman presented with 2-week duration of voice hoarseness and sore throat, associated with oral ulcers, blood-stained sputum and episodes of fever. Systems and drug reviews were unyielding. Extensive haemorrhagic ulcers and erosions were seen over the hard and soft palate, buccal mucosa, lips, tongue, pharyngeal wall and tonsillar fossae. Nasoendoscopy showed vocal cord webbing and chronic inflammatory mucosal disease (Fig. 1a). Additionally, there were multiple moist vegetative tender plaques with erosions over the patient’s scrotum, inner thighs and perineum (Fig. 1b). Histopathology of a punch biopsy of the right medial thigh nodule revealed a hyperplastic squamous epithelium with suprabasal acantholysis, free-floating keratinocytes and intraepithelial micro-abscesses filled with eosinophils and neutrophils (Fig. 2). Antibodies to desmoglein-3 were positive at 183.8 units/mL, whilst antibodies to desmoglein-1, BP180 and BP230 antibodies were negative. Immunofluorescence performed on scrotal lesions was negative for herpes simplex virus (HSV). A paraneoplastic work-up, including tumour markers and whole-body positron emission tomography–computed tomography, was normal with the exception of a positive faecal occult blood test. The patient declined colonoscopy. The diagnosis of pemphigus vegetans was made based on clinical, biochemical and histopathological findings. Systemic prednisolone 30 mg daily and topical triamcinolone 0.1% oral paste for oral ulcers was commenced. Most lesions resolved after 10 days. Repeat nasoendoscopy 8 weeks later demonstrated complete resolution of oral ulcers and vocal cord webbing. After the initial good clinical response, tapering of prednisolone was started but the patient had difficulty adhering due to cognitive impairment. He experienced a flare and required azathioprine 50 mg daily before undergoing successful prednisolone wean.