**Author details**

manifestations [63, 64]. No infectious, autoimmune, or neoplastic reason has been shown. Excessive activation of the interleukin 1 beta (IL‐1β) pathway is most commonly held

Many syndromes such as familial Mediterranean fever (FMF), Tumor necrosis factor (TNF) receptor‐associated periodic syndrome, hyperimmunoglobulinemia D with periodic fever syndrome (HIDS), and cryopyrin‐associated periodic syndromes have been identified among the autoinflammatory syndromes. A monogenic defect has been found but only in some of these disorders. However, all have been included within the autoinflammatory syndromes as

Autoinflammatory syndromes mostly start in infancy or during childhood. Although most cases are familial, some are sporadic. Recurrent episodes of inflammation with fever, eleva‐ tion in acute‐phase reactants, and skin rash can be seen in the absence of an infectious or auto‐ immune etiology. Although joint and skin involvement can be seen in various forms, fever is almost always present. These symptoms can also be accompanied by systemic findings such as abdominal pain, myalgia, ocular involvement, serositis, amyloidosis, and neurological

The skin signs show variety. Urticarial lesions are the predominant skin signs, especially in cryopyrinopathies, and occur in the first year of life. They are more commonly seen as erysipelas‐like plaques in the lower extremities in FMF. Erythematous macules and urticarial

Autoinflammatory disorders can pose a significant challenge for primary care physicians, pediatricians, dermatologists, rheumatologists, and infectious disease specialists in terms of wide‐ranging clinical spectrum. A perivascular and interstitial neutrophil‐rich infiltration suggesting neutrophilic urticarial dermatoses is observed in the histopathologic evaluation of skin lesions. Leukocytoclastic vasculitis‐like signs can also be seen [65, 66]. However, these signs are not specific. The diagnosis of autoinflammatory disorders is usually made with the clinical features and then supported by either genetic testing or the patient's response to IL‐1

Ordinary urticaria is a clinical picture frequently encountered by dermatologists and usu‐ ally presents no diagnostic difficulty. However, cutaneous and systemic urticarial syndromes should be considered in the case of persistence of urticarial lesions, bilateral and symmetri‐ cal location, healing with hyperpigmentation or bruising, the presence of other elementary lesions, not responding to systemic antihistamines, and being accompanied by systemic find‐ ings. The differential diagnosis of ordinary urticaria and urticarial syndromes is not easy. A detailed clinical evaluation should therefore be performed. Clinicopathologic correlation and, if necessary, further studies should be conducted in the presence of findings suggesting

responsible in the etiopathogenesis [64].

126 A Comprehensive Review of Urticaria and Angioedema

they show similar inflammatory features [63].

signs [63, 65].

**4. Conclusion**

urticarial syndromes.

lesions are seen in HIDS [51, 65].

inhibition or other specific therapies [63].

Hilal Gokalp1 \* and Isil Bulur2

