PMC6452561_01
(a) Oesophagogastroduodenoscopy demonstrating extensive pharyngeal ulceration. (b) Ulcerating plaque (10 cm diameter) over the left central back; photograph taken by the patient's family member prior to admission. Reproduced with patient consent.
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Several painful mouth ulcers were seen, affecting the hard palate and the oropharynx (Figure 1(a)). She had a 10 cm diameter inflammatory well-demarcated plaque over the left central back (Figure 1(b)) and smaller ulcers over the scalp, all less than 1 cm diameter. Upper gastrointestinal endoscopy revealed marked oropharyngeal ulceration without contact bleeding (Figure 1(a)).
(a) H and E stained skin punch biopsy (x20 magnification) showing loss of the epidermis with an inflammatory infiltrate in the dermis composed of neutrophils and histiocytes, the latter coalescing into (b) poorly formed granulomas (x100 magnification). Abundant cytoclastic debris is present. (c) In areas the inflammation appears centred on blood vessels associated with fibrinoid necrosis (x200 magnification). The findings indicate a necrotising vasculitis with granulomatous features.
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Immunohistochemistry from both locations revealed loss of epidermis with necrosis and a brisk inflammatory infiltrate in the dermis composed of neutrophils and histiocytes, the latter coalescing into poorly formed granulomas (Figures 2(a) and 2(b)). In areas, the inflammatory cells were clearly centred around and obscuring blood vessels, which showed foci of fibrinoid necrosis (Figure 2(c)).