Handbook of Dermatology by Margaret W. Mann & Daniel Popkin

Author:Margaret W. Mann & Daniel Popkin
Language: eng
Format: epub
ISBN: 9781118408520
Publisher: Wiley
Published: 2019-09-27T00:00:00+00:00

Hereditary periodic fever syndromes

Familial mediterranean fever/FMF: Recurrent fever (few hours to several days), recurrent polyserositis (peritoneum, synovium, and pleura), AA amyloidosis, renal failure, erysipelas‐like erythema esp. BLE, rare associations: HSP and PAN. AR or AD, MEFV/Pyrin defects

Hyper‐IgD with periodic fever/HIDS: Recurrent fever (3–7 days, 1–2 months apart), abdominal pain, diarrhea, headache, arthralgias, cervical lymphadenopathy, erythematous macules > papules and nodules, elevatated IgD and IgA, rare associations: HSP and EED, mevalonic aciduria. AR, MVK defects

TNF receptor‐associated periodic/TRAPS/Hibernian fever: Recurrent fever (usu > 5 days, often 1–3 weeks), myalgia (w/ overlying migratory erythema), pleurisy, abdominal pain, conjunctivitis/periorbital edema, serpiginous, edematous, purpuric, or reticulated lesions esp. at extremities, AA amyloidosis, renal failure, leukocytosis, elevated ESR. AD, TNF‐Receptor 1 defects

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