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Allergic and Immunologic Reactions
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Saira N. Agarwala, Aspen R. Trautz, Sylvia Hsu
Differential diagnosis: Urticarial vasculitis is often indistinguishable from urticaria, but lesions last greater than 24 hours. The differential includes urticaria pigmentosa, familial cold urticaria, bradykinin-mediated angioedema (e.g., hereditary angioedema), and urticarial pemphigoid.
Phagocytic cells and their functions
Published in Gabriel Virella, Medical Immunology, 2019
Gabriel Virella, John W. Sleasman
Familial cold autoinflammatory syndrome (FCAS), also known as familial cold urticaria, is a mild form of CAPS typically triggered by cold exposure and characterized by febrile urticarial rash with headache, arthralgia, and occasionally conjunctivitis, but no central nervous system symptoms.
Systemic Physical Condition
Published in A. Sahib El-Radhi, Paediatric Symptom and Sign Sorter, 2019
Periodic fever (PF) and relapsing fever (ReF) are characterized by episodes of fever recurring at regular or irregular intervals; each episode is followed by one to several days, weeks or months of normal temperature. Examples are seen in malaria, brucellosis, familial Mediterranean fever (FMF) and PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis). ReF is recurrent fever caused by numerous species of Borrelia and transmitted by lice (louse-borne ReF) or ticks (tick-borne ReF). Familial cold urticaria syndrome (FCUS), Muckle–Wells syndrome (MWS), TNF-receptor associated periodic syndrome (TRAPS) and hyperimmunoglobulinemia D syndrome (HIDS) are also characterized by recurrent episodes of fever but they are rare compared with FMF.
Familial cold autoinflammatory syndrome with rheumatoid arthritis
Published in Baylor University Medical Center Proceedings, 2021
Brooke Walterscheid, Jeannie Nguyen, Swetha Gadwala, Goutam Shome, Michelle Tarbox, James A. Tarbox
FCAS, also known as familial cold urticaria or familial polymorphous cold eruption, is a rare autosomal dominant disorder and is frequently misdiagnosed as chronic urticaria.6 Most cases present with self-limited episodes of fever with synovial inflammation and cutaneous manifestations lasting <24 hours.5 Cutaneous findings involve urticarial wheals recurring in either symmetrically distributed crops mimicking urticarial vasculitis or in an annular configuration with a peripheral halo of vasoconstriction. Though the trunk is favored, lesions may involve the lower and upper extremities as well.6 The characteristic association with cold exposure and onset of symptoms is unique to FCAS, distinguishing it from others in the cryopyrin-associated periodic syndrome spectrum.
Chronic spontaneous urticaria or autoinflammatory disease? The therapeutic effect of omalizumab in a pediatric patient
Published in Journal of Dermatological Treatment, 2018
Maria Cristina Maggio, Anna Lucania, Mirella Collura, Giovanni Corsello
Familial cold autoinflammatory syndrome (FCAS), also known as familial cold urticaria, is a genetic condition characterized by recurrent episodes of fever, skin rash, and joint pain after exposure to cold temperatures. These episodes usually begin in infancy and occur throughout life. FCAS is a clinical phenotype of chronic autoinflammatory periodic syndrome (CAPS) (3). An autoinflammatory disease (AID) classically characterized by recurrent intermittent episodes of rash, fever, significant increase of inflammatory markers (serum amyloid A protein [SAA], C reactive protein [CRP], erythrocyte sedimentation rate [ESR], neutrophil leukocyte count), conjunctivitis, arthralgia, myalgia, abdominal, and chest pain; affected patients can have severe clinical manifestations, and require treatment with interleukin-1 inhibitors (4).
Off-Label studies on anakinra in dermatology: a review
Published in Journal of Dermatological Treatment, 2022
Kyle Tegtmeyer, Giancarlo Atassi, Jeffrey Zhao, Nolan J. Maloney, Peter A. Lio
Additional trials are currently underway investigating additional potential dermatologic uses of anakinra, detailed on ClinicalTrials.gov. Table 5 details four ongoing trials (involving the use of anakinra in Familial Cold Urticaria, pustular skin diseases, CAPS, and Schnitzler Syndrome) for which data has not yet been published.