P19 Diagnostic conundrums in paraneoplastic pemphigus

Abstract Paraneoplastic pemphigus (PNP) is a subset of pemphigus, group autoimmune blistering skin disorders. A 51-year-old man presented with paraneoplastic associated hepatic neoplasm. He described an epigastric mass and mucositis affecting the orogenital mucosae, as well sclerae. Both computed tomography magnetic resonance imaging demonstrated 14-cm arising from left lobe liver. liver biopsy showed features atypical spindle-cell neoplasm, but definite diagnosis was not possible. diagnostic taken his outer lip mucosae. Histology surface ulceration acute inflammatory infiltrate, lichenoid lymphoplasmacytic infiltrate focal basal vacuolar change, degenerate keratinocytes adjacent suprabasal acantholysis spongiosis. Perilesional direct immunofluorescence (DIF) negative; however, indirect (IIF) intercellular IgG antibodies on transitional epithelium high titre 1 : 800 to rat bladder. These finding were consistent suspected clinical pemphigus. The patient commenced oral prednisolone mg kg−1 daily. Topical therapy included budesonide mixed sweetener applied Following this, trial 3 days intravenous methylprednisolone along 2% cocaine mouthwash, triple mouthwash (betamethasone, doxycycline nystatin) Dermovate® ointment commenced, improvement noted in pain, haemorrhage ulceration. Intravenous immunoglobulin administered preoperatively. subsequently transferred specialist centre for hepatobiliary surgery listed debulking mass. Postoperatively, he had plasmapheresis, reduction mucosal significant pain. Chemotherapy doxorubicin ifosfamide initiated thereafter. PNP severe disorder characterized clinically by flaccid blisters erosions. Although much yet be understood regarding pathophysiology PNP, studies show that development autoantibodies plakin family desmogleins play particular role its pathogenesis. In addition autoantibodies, CD8+ T cells have also been shown contribute PNP. adults, majority cases are underlying haematological malignancy and, rarely, solid-organ tumour. DIF findings include C3 deposition basement membrane zones, while IIF detects proteins found or stratified squamous epithelium. As our case, reports can negative, one study showing positive only 41% cases. This case highlights complex treatment challenges practitioners face when dealing patients it affects.