[IgA pemphigus of the subcorneal pustular dermatosis type. Successful therapy with a combination of dapsone and acitretin]

Abstract

IgA pemphigus of the subcorneal pustular dermatosis type is a rare autoimmune blistering disease in the pemphigus spectrum. Patients are clinically characterized by extensive erythemas that primarily affect intertriginous areas. The erythematous macules are covered with numerous vesicles and pustules with occasional hypopyon formation. Histopathology shows subcorneal acantholysis with clefting and numerous neutrophils within the blister as well as in the edematous papillary dermis. IgA autoantibodies bind in vivo to keratinocytes within the upper half of the epidermis. Desmocollin 1, the autoantigen of this disease, is a member of desmosomal cadherins and is only expressed on more differentiated keratinocytes. The demonstration of circulating autoantibodies against desmocollin 1 in routine diagnosis is challenging and requires indirect immunofluorescence staining of desmocollin 1 transfected COS7 cells. We report a patient with a severe course of the disease who only responded to combined therapy with dapsone and acitretin.