Diagnosis and Management of Acute and Chronic Urticaria

Acute urticaria and angioedema are differentiated from chronic urticaria and angioedema (CUA) based on duration of illness. ( D )

Acute urticaria and angioedema should be differentiated from anaphylaxis. ( D )

Acute urticaria and angioedema are more frequently associated with identifiable conditions. When this disorder becomes chronic, it is less likely to be associated with an identifiable cause. ( D )

Acute urticaria and angioedema are often but not always related to mast cell and basophil activation from multiple triggers, which include IgE- and non–IgE-mediated mechanisms. ( LB )

Although skin biopsy is not indicated in most cases of acute urticaria and angioedema, it might occasionally be useful for differentiating this condition from other inflammatory disorders. ( C )

Common causes of acute urticaria and angioedema, including medications and foods, should be identified by a detailed history and eliminated, if possible. ( C )

Cryoglobulinemia is often found in many conditions that result in vasculitis. ( D )

Autoinflammatory syndromes are a group of conditions that involve aberrant activation of mediators of the innate immune response with resultant fever and other symptoms. (Table 5) ( C )

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). (Table 5) ( C )

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. (Table 5) ( C )

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 )

Swelling of the area in the medial portion of the upper eyes might be a sign of thyroid orbitopathy and misinterpreted as angioedema. (Table 6) ( C )

Urticaria-like dermatoses can occur at various stages of pregnancy. (Table 6) ( C )

Women who present with cyclical urticaria can have autoimmune progesterone-induced dermatitis. (Table 6) ( C )

Episodic attacks of angioedema with weight gain are characteristic of the syndrome episodic angioedema with eosinophilia (Gleich syndrome). ( C )

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 )

Cutaneous mast cell disorders that can present with urticaria-like lesions include urticaria pigmentosa (UP), mastocytomas, and telangiectasia macularis eruptiva perstans (TMEP). ( C )

Erythema multiforme can resemble urticaria and might be caused by viral infections (e.g., herpes), mycoplasma infection, or medications. ( C )

Hepatitis B or C can be associated with urticarial vasculitis and should be considered in the differential diagnosis, particularly for patients whose behaviors predispose to contracting a sexually transmitted disease, who have recently received a blood transfusion, or who have exposure to contaminated needles. ( C )

Bullous pemphigoid can present initially with urticaria-like papules or small plaques that can be excoriated by the patient before noticeable blistering occurs. (Table 7) ( D )

Persistent swelling of the lips without evidence of eczematous dermatitis might be a sign of cheilitis granulomatosa (Melkersson-Rosenthal syndrome). ( C )

Polymorphous light eruption differs from solar urticaria in that onset usually occurs minutes to hours after sunlight exposure, and the eruption lasts for days compared with solar urticaria, which is short-lived between exposures. ( D )

Recall urticaria is a condition in which urticaria is observed at the site of a previous sting or injection after re-exposure to the same inciting factor. ( C )

Patients with Schnitzler syndrome caused by an IgM monoclonal gammopathy present with nonpruritic urticaria (that spares the face), bone pain, and intermittent fever. ( D )

Hepatitis B or C can be associated with urticarial vasculitis and should be considered in the differential diagnosis, particularly for patients whose behaviors predispose to contracting a sexually transmitted disease, who have recently received a blood transfusion, or who have exposure to contaminated needles. ( C )

Bullous pemphigoid can present initially with urticaria-like papules or small plaques that can be excoriated by the patient before noticeable blistering occurs. ( D )

Persistent swelling of the lips without evidence of eczematous dermatitis might be a sign of cheilitis granulomatosa (Melkersson-Rosenthal syndrome). ( C )

Polymorphous light eruption differs from solar urticaria in that onset usually occurs minutes to hours after sunlight exposure, and the eruption lasts for days compared with solar urticaria, which is short-lived between exposures. ( D )

Recall urticaria is a condition in which urticaria is observed at the site of a previous sting or injection after re-exposure to the same inciting factor. ( C )

Patients with Schnitzler syndrome caused by an IgM monoclonal gammopathy present with nonpruritic urticaria (that spares the face), bone pain, and intermittent fever. ( D )