Paraneoplastic pemphigus: Diagnostic mimics, confounders and management challenges in a series of five long-term survivors from a single centre

副肿瘤性天疱疮:来自同一中心的五例长期生存者的诊断相似症、混杂因素和治疗挑战

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Abstract

We present a series of five cases who presented to our institution with treatment-refractory mucosal ulceration, all of whom were subsequently diagnosed with paraneoplastic pemphigus (PNP). This case series highlights the diagnostic and treatment considerations for PNP - in particular, the steroid-dependent, recalcitrant, polymorphic manifestations; the combination of histopathological and clinical findings that may overlap with clinically similar diseases, for example, pemphigus vulgaris and lichen planus; the importance of immunopathological findings for its diagnosis, and the need for surveillance and management of life-threatening bronchiolitis obliterans.

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