Familial hibernian fever; TRAPS
HistoryThis section has been translated automatically.
Bouroncle and Doan, 1957; Williamson et al., 1982
DefinitionThis section has been translated automatically.
Hereditary, inflammatory disease characterized by recurrent episodes of fever combined with abdominal discomfort, arthralgia, conjunctivitis and periorbital edema as well as a generalized maculo-papular exanthema.
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EtiopathogenesisThis section has been translated automatically.
Autosomal-dominantly inherited mutations of the TNFRSF1A gene (gene locus: 12p13.2) encoding the 55 kDa TNF receptor.
ManifestationThis section has been translated automatically.
Beginning of the first clinical manifestations is possible in the first weeks of life; late manifestations in adulthood after the age of 25 have been described; women are affected about twice as often as men.
LocalizationThis section has been translated automatically.
Located mainly on the trunk and extremities.
Clinical featuresThis section has been translated automatically.
HistologyThis section has been translated automatically.
Superficial and deep lymphocytic perivascular dermatitis.
Differential diagnosisThis section has been translated automatically.
Familial Mediterranean fever; hyperimmunoglobulin D syndrome.
TherapyThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Bouroncle BA, Doan CA (1957) 'Periodic fever': occurrence in five generations. On J Med 23: 502-506
- Drenth JPH, van der Meer JWM (2001) Hereditary periodic fever. New Eng J Med 345: 1748-1757
- Kusuhara K et al (2004) Tumour necrosis factor receptor-associated periodic syndrome with a novel mutation in the TNFRSF1Agene in a Japanese family. Eur J Pediatr 163: 30-32
- Takada K et al (2003) Favorable preliminary experience with etanercept in two patients with the hyperimmunoglobulinemia D and periodic fever syndrome. Arthritis Rheum 48: 2645-2651
- Toro JR (2000) Tumor Necrosis Factor Receptor-Associated Periodic Syndrome. Arch Dermatol 136: 1487-1494
- Williamson LM, Hull D, Mehta R, Reeves WG, Robinson BHB, Toghill PJ (1982) Familial hibernian fever. Quart J Med 51: 469-480
Incoming links (6)Dermatitis-arthritis syndromes; Familial cold urticaria; Mediterranean fever, familial; Pfapa syndrome; TNFRSF1A Gene; Traps;
Outgoing links (6)Dermatitis; Etanercept; Glucocorticosteroids; Non steroidal anti-inflammatory drugs; Plaque; Rash;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.