Table 2.

Resume of PNP diagnostic algorithm.

CLINIC	Bullous Lesions on Skin and Mucous Membranes
PATHOLOGY	Acantholysis (intraepidermal bulla)	Sub-epidermal cleavage
DIF	Combined presence of IgG and complement (C3) granular-linear deposition within the epidermal intercellular spaces and along the basement-membrane zone
IIF	Presence of circulating antibodies that target the intercellular zone of stratified squamous or transitional epithelia
LABORATORY	AAB directed to several proteins, including desmoplakin I (250 kD), bullous pemphigoid antigen (230 kD), envoplakin (210 kD), desmoplakin II (210 kD), periplakin (190 kD) and α-2-macroglobulin-like-1 (170 kD) IgG anti-DSG 1 and 3 IgG anti-DSG 1 IgA anti-DSC
DIAGNOSIS	PNP PV PF IgAP	Exclusion of PNP

Abbreviations: PNP Paraneoplastic pemphigus; DIF Direct immunofluorescence; IIF Indirect immunofluorescence; AAB Autoantibodies; PV Pemphigus vulgaris; PF Pemphigus foliaceus; IgAP IgA pemphigus.