Diagnostic criteria - all of the following:
(1) presence of systemic symptoms (fever, malaise, other)
(2) erythematous areas with multiple, isolated aseptic pustules over a large portion of the skin surface
(3) biopsy of the lesions shows Kogoj's spongiform pustules on microscopic examination
(4) some of the following laboratory findings:
(4a) leukocytosis with left shift
(4b) increased erythrocyte sedimentation rate (ESR)
(4c) elevated C-reactive protein
(4d) elevated anti-streptolysin O (ASO) antibody titer
(4e) increased concentrations of IgG and IgA
(4f) hypoproteinemia
(4g) hypocalcemia
(5) recurrent episodes of the above findings
Exclusions:
(1) pustules developing in psoriasis vulgaris following therapy with or following withdrawal of corticosteroids or other drugs
(2) any form of psoriasis without systemic features (such as circinate annular form)
(3) subcorneal pustular dermatosis
Inclusions:
(1) impetigo herpetiformis
(2) generalization of acrodermatitis continua
(3) juvenile or infantile forms of GPP
