Multiple cutaneous squamous cell carcinomas with confluent noncaseating epithelioid granulomas in PLCG2-associated antibody deficiency and immune dysregulation (PLAID) syndrome: A case report

Phospholipase-C γ2-associated antibody deficiency and immune dysregulation (PLAID) is a rare, genetically complex immunodeficiency syndrome characterized by cold urticaria, noninfectious cutaneous granulomas, autoimmunity, and humoral immune deficiency. The syndrome was only recently discovered after linkage analy sis of three families with lifelong cold-induced urticaria and variable immune defects revealed dominantly inherited genomic deletions resulting in the temperature-sensitive constitutive activation of PLCG2, which encodes a signaling molecule expressed in B cells, natural killer cells, and mast cell s.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research