Pyoderma gangrenosum (PG) is an orphan disease. While research on such disorders 
is based on only few randomized multicenter as well as retrospective studies, 
most of the data comes from case series of small patient groups. Apart from 
topical and intralesional therapeutic options for early stages and mild disease 
courses, treatment predominantly involves systemic therapeutic agents. Besides 
systemic corticosteroids and cyclosporine A (CsA), options also include 
intravenous immunoglobulins (IVIG) and biologics such as the TNFα inhibitors 
infliximab, adalimumab, and etanercept; the interleukin (IL) 12/23 antibody 
ustekinumab; the IL-1 receptor antagonist anakinra; and the IL-1β antibody 
canakinumab. The best evidence-based study data is available for CsA, 
prednisolone, and infliximab; the latter especially in patients with concomitant 
ulcerative colitis or Crohn's disease. A response to IVIG and canakinumab has 
been reported in smaller case series. First described by Brocq almost 100 years 
ago, it was soon recognized that PG did in fact require treatment. To this day, 
however, such treatment remains a clinical challenge. Despite the severe - 
albeit rare -clinical picture, improvement in therapeutic options may be 
expected in the future, primarily due to further clinical studies - especially 
with a greater number of patients, a better understanding of the 
etiopathogenesis, as well as the use of modern targeted therapies with higher 
efficacy and a lower rate of side effects than conventional immunosuppressants 
such as prednisolone and CsA.