Table 1

Conditions to consider in the differential diagnosis of urticaria.

Urticarial vasculitis

• Lesions are usually painful (rather than pruritic), last >48 hours, and leave discoloration on the skin

Systemic mastocytosis

• Rare condition that involves the internal organs (liver, spleen, lymph nodes, bone marrow), in addition to the skin

Atopic dermatitis

• Chronic, highly pruritic inflammatory skin disease

• Clinical manifestations vary with age

Bullous pemphigoid

• Chronic, autoimmune, blistering skin disease

Erythema multiforme

• Acute, self-limited, skin condition

• Considered to be a type IV hypersensitivity reaction to certain infections, medications, and other various triggers

Familial cold autoinflammatory syndrome

• Rare, inherited inflammatory disorder characterized by recurrent episodes of rash, fever/chills, joint pain, and other signs/symptoms of systemic inflammation triggered by exposure to cooling temperatures

• Onset usually occurs during infancy and early childhood and persists throughout the patient’s life

Fixed drug eruptions

• Lesions occur from exposure to a particular medication and occur at the same site upon re-exposure to the offending medication

• Lesions usually blister and leave residual pigmentation

Subacute cutaneous lupus erythematosus

• A non-scarring, photosensitive skin condition

• May occur in patients with systemic lupus erythematosus (SLE) and Sjögren syndrome

Pruritic urticarial papules and plaques of pregnancy

• Benign skin condition that usually arises late in the third trimester of a first pregnancy

Muckle-Wells syndrome

• Rare genetic disease that causes hearing loss and recurrent hives

• May lead to amyloidosis

Schnitzler's syndrome with monoclonal IgG kappa gammopathy

• Rare disease characterized by chronic, non-pruritic hives, periodic fever, bone and joint pain, swollen lymph glands and an enlarged spleen and liver

Kanani et al. Allergy, Asthma & Clinical Immunology 2011 7(Suppl 1):S9   doi:10.1186/1710-1492-7-S1-S9