Table 2 Differential diagnosis of chronic urticaria
From: Management of chronic urticaria in children: a clinical guideline
Disease | Clinical criteria | Laboratory/instrumental data | Diagnostic criteria |
|---|---|---|---|
-Maculae, round or oval papulae, brownish, from few mm to 2 cm diameter. Lesions become erythematous and swollen after mechanical stimulation (Darier’s sign). -Asymptomatic, rarely mild itch. -Systemic forms are associated with flushing, wheezing, abdominal pain, diarrhoea, syncope. | -Possible elevated serum tryptase in intercritical phase (systemic mastocytosis). -Possible eosinophilia (cutaneous and systemic mastocytosis). -Possible cytopenia (systemic mastocytosis). | -History. -Clinical features; Darier’s sign. -In rare cases, skin biopsy. | |
Papulous urticaria (strophulus; children papulous dermatitis) [130, 131] | -Erythematous papulae of few mm diameter on exposed areas (face, limbs), isolated or confluent, sometimes with vesicles on top, rarely itchy. Long persistence. Spontaneous resolution. [128] | None. | -History, contact with agents in gardens, fields, animals etc. -Clinical features. |
Vasculitic urticaria [132, 133] Normocomplementemic | -Papulous erythemato-purpuric lesions that do not fade with finger pressure, lasting over 24 h. Pain and/or burning feeling, sometimes itch. It can be associated with fever, arthralgia, petechiae. Lesions resolve with secondary hyperpigmentation. | -Blood cell count. -Increase of inflammatory markers, ANA, anti-DNAds antibody, rheumatoid factor positivity. -Mutation of IL3 DNASE. | -History. -Skin biopsy (leukocytoclastic vasculitis). |
-Fever, arthralgia, petechiae. -Association with systemic lupus erythematosus. | - Hypocomplementemia (C1q, C3, C4). -Elevated ESR. -Positive ANA, anti-DNAds antibody, rheumatoid factor. | History. Urticaria for over 6 months. -Systemic symptoms. -Skin biopsy (leukocytoclastic vasculitis). | |
Mc Duffie syndrome with anti-C1q antibodies | -Urticaria for over 6 months associated with arthritis, arthralgia, lung pathology, uveitis, episcleritis, glomerulonephritis. | -C1q, C3, C4; anti-C1q autoantibodies. | -History. -Urticaria for over 6 months. -Skin biopsy (leukocytoclastic vasculitis). -Anti-C1q autoantibodies. |
Criopirinopathy (CAPS) [134]: 3 different phenotypes -Familial cold autoinflammatory syndrome (FCAS) -Muckle-Wells syndrome -Neonatal onset multisystemic inflammatory disorder (NOMID) or chronic infantile neuro-cutaneous articular syndrome (CINCA) | -Autosomic dominant, de novo mutations described. -Early onset in the first months of life. -Plaques, erythema or papulae that disappear in 24 h; no itch. -Exanthema, fever, arthralgia and conjunctivitis after 1–2 h of exposure to cold, lasting < 24 h. -Arthralgia or periodic arthritis, conjunctivitis, secondary generalized amyloidosis, neurosensory deafness. -Maculo-papular wheal-like eruption, non-constant fever, failure to thrive, neurosensory progressive hypoacusis, uveitis, optic neuritis to blindness, variable articular symptoms, non-foreseeable defects of long bone growth, chronic meningitis, chronic headache, intellectual disability. | -Increased ESR, CRP, anaemia, neutrophilic leucocytosis, absence of autoantibodies. -Negative response to cold challenge. | -More than 2 markers among: urticarial rash; exacerbations with cold/stress; neurosensory hypoacusis; arthralgia/arthritis, myalgia; chronic aseptic meningitis; skeletal abnormalities (overgrowth of epiphysis and frontal bone) WITH: increase of inflammation markers; serum A amyloid. -Molecular diagnosis (NLRP3, NLRP12); |
Tumor necrosis factor (TNF) receptor 1 associated periodic syndrome (TRAPS) | -Neutrophilic leucocytosis, increased ESR and CRP, A amyloid serum; soluble TNF receptor. | -History. Mutation of the TNFRSF1A gene. | |
Bradykinin mediated angioedema [135] | -Dominant autosomic or de novo mutation. -Isolated angioedema lasting more than 24 h; no itch; sometimes gastrointestinal or respiratory involvement. -No response to anti-histamines. -Two different types, a most frequent one caused by C1-INH quantitative defect, the other by functional impairment. | -Decrease of C4 levels. -Reduced C1-INH in the first type. -C1-INH and C4 levels reach adult levels at 2–3 years old. | -History. Intake of ACE inhibitors. -C4 determination. -Quantitative and functional determination of C1 INH. -Mutations of SERPING 1 gene. |
Hypoproteinemic oedema | -Peripheral swelling, serous cavities effusion. Diarrhoea, poor growth. Nutritional deficit. | Serum protein electrophoresis. | -History. -Systemic symptoms. -Hypoalbuminemia. |
Head and neck tumors, lymphoma | Local swelling. | -Imaging (RX, CT, MRI). -ESR, LDH, Serum protein electrophoresis. | -History. -Biopsy. |