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Urticaria

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14 Differential Diagnosis (Table 4) Î Cryoglobulinemia is often found in many conditions that result in vasculitis. (D) Î Autoinflammatory syndromes are a group of conditions that involve aberrant activation of mediators of the innate immune response with resultant fever and other symptoms. (C) (Table 5) Î Cryopyrin-associated periodic syndromes (also referred to as cryopyrinopathies) are a group of autoinflammatory syndromes that are characterized by abnormalities in the C1AS1 gene, which encodes for the cryopyrin protein, and are associated with an urticaria-like rash (pseudourticaria). (C) (Table 5) Î Hypocomplementemic or normocomplementemic urticarial vasculitis is associated with decreased or normal complement (C1q, C4, and C3) levels and a biopsy that reveals vasculitis of dermal blood vessels with leukocytoclasis. (C) (Table 5) Î Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a more severe form of this condition associated with arthralgias, glomerulonephritis, uveitis or episcleritis, recurrent abdominal pain, obstructive lung disease, and urticaria and/or angioedema. (C) Î Swelling of the area in the medial portion of the upper eyes might be a sign of thyroid orbitopathy and misinterpreted as angioedema. (C) (Table 6) Î Urticaria-like dermatoses can occur at various stages of pregnancy. (C) (Table 6) Î Women who present with cyclical urticaria can have autoimmune progesterone-induced dermatitis. (C) (Table 6) Î Episodic attacks of angioedema with weight gain are characteristic of the syndrome episodic angioedema with eosinophilia (Gleich syndrome). (C) Î Hypereosinophilic syndrome (HES) should be considered when the peripheral total eosinophil count exceeds 1500/mL for >6 months in the absence of other causes of peripheral eosinophilia. (C) Î Cutaneous mast cell disorders that can present with urticaria-like lesions include urticaria pigmentosa (UP), mastocytomas, and telangiectasia macularis eruptiva perstans (TMEP). (C) Mast cell activation disorders can also present with urticaria and angioedema but usually have additional systemic symptoms. (C) (Table 7) Î Erythema multiforme can resemble urticaria and might be caused by viral infections (e.g., herpes), mycoplasma infection, or medications. (C) Differential Diagnosis

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