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Intraepidermal neutrophilic type (IEN) Desmocollin 1 is the main antigen target overall particularly in the SPD sub-type Dsg 1 and 3 can be targeted also Both types associated with intercellular IgA deposits on immunoflourescence There is NO ACANTHOLYSIS on histology (unlike pemphigus vulgaris, pemphigus foilaceous and paraneoplastic pemphigus) Clinical: SPD clinically presets as serpiginous vesicles and pustules IEN classically presents as flaccid pustules and bullae arranged in an annular, polycyclic (sometimes referred as sun-flower like) arrangements usually in intertriginous areas The pustules tend to quickly rupture and you get an annular crust over the plaque Commonly affects flexural areas like axilla and groin but can be more widespread Associations: Monoclonal IgA gammopathy Multiple myeloma Others reported: HIV, Sjogrens, RA, Crohn’s Investigations: H&E: Intra-epidermal neutrophillic infiltration Little to no acantholysis - have mild loss of cohesion between keratinocytes Subcorneal pustular dermatosis subtype - increased intensity of IgA autoantibodies in the upper surface of the epidermis

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