Overview of Hereditary Periodic Fever Syndromes

Familial Mediterranean fever Familial Mediterranean Fever Familial Mediterranean fever is a hereditary disorder characterized by episodes of high fever with abdominal pain or, less commonly, chest pain, joint pain, or a rash. Familial Mediterranean... read more

PFAPA syndrome PFAPA Syndrome PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome causes recurring episodes of fever that last 3 to 6 days, mouth sores (stomatitis), a sore throat (pharyngitis)... read more (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis)

Hereditary cryopyrin-associated periodic syndromes (cryopyrinopathies): These syndromes include familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem autoinflammatory disease (NOMID). Episodes of fever, a rash, and joint pain are periodically triggered by cold temperatures. The drugs anakinra, rilonacept, or canakinumab, which modify how the immune system functions, may help.

Hyper-IgD syndrome: This syndrome causes fever, abdominal pain, headache, joint pain, a rash, and swollen lymph nodes. nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and anakinra may help relieve symptoms during attacks. Anakinra or canakinumab is given to prevent attacks.

Tumor necrosis factor receptor–associated periodic syndrome (TRAPS): This syndrome causes periodic attacks of muscle pain and swelling in the arms and legs, abdominal pain, joint pain, and rash in addition to fever. Prednisone, a corticosteroid, is used to suppress symptoms during attacks. Anakinra, canakinumab, or sometimes etanercept are used for attacks.

PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum Pyoderma Gangrenosum Pyoderma gangrenosum is a chronic inflammatory skin disorder of unknown cause that causes large sores on the skin. (See also Overview of Hypersensitivity and Reactive Skin Disorders.) The cause... read more , and acne Acne Acne is a common skin condition causing pimples and other abnormalities on the face and upper torso. Acne is caused by a buildup of dead skin cells, bacteria, and dried sebum that blocks the... read more ): This syndrome causes inflamed joints, skin ulcers, and acne. By puberty, the arthritis tends to subside and the skin problems are the main symptoms. Anakinra or etanercept may help. Acne Treatment Acne is a common skin condition causing pimples and other abnormalities on the face and upper torso. Acne is caused by a buildup of dead skin cells, bacteria, and dried sebum that blocks the... read more is treated with the antibiotic tetracycline or with isotretinoin.