The care pathway for children with urticaria, angioedema, mastocytosis

Cutaneous involvement characterized by urticarial lesions with or without angioedema and itch is commonlyobserved in routine medical practice. The clinical approach may still remain complex in real life, because severaldiseases may display similar cutaneous manifestations. Urticaria is a common disease, characterized by the suddenappearance of wheals, with/without angioedema. The term Chronic Urticaria (CU) encompasses a group ofconditions with different underlying causes and different mechanisms, but sharing the clinical picture of recurringwheals and/or angioedema for at least 6 weeks. Hereditary Angioedema (HAE) is a rare disorder characterized byrecurrent episodes of non-pruritic, non-pitting, subcutaneous or submucosal edema affecting the extremities, face,throat, trunk, genitalia, or bowel, that are referred as “attacks”. HAE is an autosomal dominant disease caused by adeficiency of functional C1 inhibitor, due to a mutation in C1-INH gene (serping 1 gene) characterized by the clonalproliferation of mast cells, leading to their accumulation, and possibly mediator release, in one or more organs.In childhood there are two main forms of mastocytosis, the Systemic and the Cutaneous. The clinical featuresof skin lesions in urticaria, angioedema and mastocytosis may differ depending on the aetiologic factors, and theunderlying pathophysiological mechanisms. The diagnostic process, as stepwise approach in routine clinical practice, is here reviewed for CU, HAE and mastocytosis, resulting in an integrated method for improved management of thesecutaneous diseases. Taking into account that usually these conditions have also a relevant impact on the qualityof life of children, affecting social activities and behavior, the availability of care pathways could be helpful in disentanglethe diagnostic issue achieving the most cost-effective ratio.