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Integument: Exanthema with smallest to medulla-sized, raised petechiae, later changing into brown-yellow spots.
In more severe courses, lentil- to coin-sized, reddish to blue-red, later hemorrhagic, itchy, possibly urticarial spots appear. Transition to red urticarial papules, vesicles, hemorrhagic blisters.
Possibly therapy-resistant, superinfected ulcerations (especially pretibially localized).
Frequent recurrent bacterial skin infections, especially furunculosis and abscesses.
Extracutaneous manifestations: Corresponding to primary chronic polyarthritis. Also, lymph node swelling, splenomegaly, hypersplenism with consecutive leukopenia, subfebrile temperatures. Increased bacterial infections of the upper respiratory tract.
Frequent evidence of rheumatoid nodules.
LaboratoryThis section has been translated automatically.
Leukocytopenia with relative lymphocytosis, possibly neutropenia.
Rheumatoid factor is strongly positive.
ANA frequently positive
HLA-DR4 positive in 90% of patients.
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LiteratureThis section has been translated automatically.
- Bowman SJ (2002) Hematological manifestations of rheumatoid arthritis. Scand J Rheumatol 31: 251-259
- Coakley G et al. (2000) Major histocompatibility complex haplotypic associations in Felty's syndrome and large granular lymphocyte syndrome are secondary to allelic association with HLA-DRB1 *0401. Rheumatology (Oxford) 39: 393-398
Ditzel HJ et al. (2000) Cloning and expression of a novel human antibody-antigen pair associated with Felty's syndrome. Proc Natl Acad Sci U S A 97:9234-9239.
- Felty AR (1924) Chronic arthritis in the adult, associated with splenomegaly and leucopenia. A report of 5 cases of an unusual clinical syndrome. Bulletin of the Johns Hopkins Hospital (Baltimore) 35: 16
- Hellmich B (2002) Autoantibodies against granulocyte colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus erythematosus. Arthritis Rheum 46: 2384-2391
- Hellmich B et al. (2002) Low Fcgamma receptor III and high granulocyte colony-stimulating factor serum levels correlate with the risk of infection in neutropenia due to Felty's syndrome or systemic lupus erythematosus. Am J Med 113: 134-139
- Robertson LP (2000) Generalized morphoea in a patient with Felty's syndrome. J Eur Acad Dermatol Venereol 14: 191-193
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Incoming links (5)
EEF1A1 gene; Large granular lymphocytic Leukemia; Neutropenia; Rheumatoid arthritis and skin manifestations; Still-chauffard raymond syndrome;Outgoing links (6)
Abscess; Adult onset still disease; Furunculosis; Rheumatic nodules; Rheumatoid arthritis and skin manifestations; Sjögren's syndrome;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer
