title: Disseminated pustular dermatosis in polycythemia vera. Relationship with neutrophilic dermatosis of myeloproliferative disorders: study of neutrophil function.
abstract: An unusual case of disseminated pustular eruption associated with polycythemia vera is described. This eruption can be included in the group of neutrophilic dermatoses of myeloproliferative disorders (pyoderma gangrenosum, Sweet's syndrome). A defect in superoxide anion (O2-) generation is detected. The fact that both the clinical condition and the neutrophil function improved with the use of dapsone suggests that the O2- generation defect may play a role in the pathogenesis of the eruption. From an evolutionary standpoint, the occurrence of a neutrophilic dermatosis in polycythemia vera could be an unfavorable sign of preleukemia.
authors.affiliation: Hôpital Ste. Marguerite, Marseille, France.
authors.forename: J J
authors.initials: JJ
authors.lastname: Grob
authors.forename: J L
authors.initials: JL
authors.lastname: Mege
authors.forename: A M
authors.initials: AM
authors.lastname: Prax
authors.forename: J J
authors.initials: JJ
authors.lastname: Bonerandi
mesh_terms: D000368:Aged; D003622:Dapsone / Q000627:therapeutic use; D006801:Humans; D066298:In Vitro Techniques; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology; D009240:N-Formylmethionine Leucyl-Phenylalanine / Q000494:pharmacology; D009504:Neutrophils / Q000187:drug effects / Q000378:metabolism; D011087:Polycythemia Vera / Q000150:complications*; D012867:Skin / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D013280:Stomatitis / Q000209:etiology / Q000473:pathology; D013481:Superoxides / Q000378:metabolism; D013755:Tetradecanoylphorbol Acetate / Q000494:pharmacology
chemical_list: D013481:Superoxides; D009240:N-Formylmethionine Leucyl-Phenylalanine; D003622:Dapsone; D013755:Tetradecanoylphorbol Acetate