Disseminated pustular dermatosis in polycythemia vera. Relationship with neutrophilic dermatosis of myeloproliferative disorders: study of neutrophil function.
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.
Grob
J J
JJ
Hôpital Ste. Marguerite, Marseille, France.
Mege
J L
JL
Prax
A M
AM
Bonerandi
J J
JJ
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
D013481:Superoxides; D009240:N-Formylmethionine Leucyl-Phenylalanine; D003622:Dapsone; D013755:Tetradecanoylphorbol Acetate