Chart 5.

Bipolar aphthosis as skin manifestation in various autoinflammatory syndromes

Disease	Possible skin lesion	Systemic involvement	Therapeutic options
Behçet’s disease	Oral and genital aphthous ulcers, erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform lesions and erythema nodosum	Uveitis, amaurosis, large vessel vasculitis, cerebrovascular and gastrointestinal involvement and pulmonary vascular disease	Corticosteroid, colchicine, azathioprine, methotrexate, cyclosporine, thalidomide, anakinra
Periodic fever, aphthous pharyngitis and cervica lymphadenopathy (PFAPA)	Oral and genital aphthous ulcers and less commonly erythema on the trunk, palmoplantar macules or purpura, acneiform lesions and pyoderma gangrenosum	Pharyngitis, and cervical lymphadenopathy, headache, abdominal pain, nausea, vomiting, myalgia, arthritis, arthralgia, splenomegaly, slight increase in IgM, IgD and IgA, leukocytosis and slight increase in acute phase proteins	Corticosteroid, cimetidine, colchicine, anakinra, tonsillectomy and adenoidectomy