ABSTRACT
Hyperzincemia and hypercalprotectinemia is a rare inflammatory disease caused by a mutation in the PSTPIP1 gene, with a dysregulation of calprotectin metabolism. Calprotectin is a zinc-binding protein with antimicrobial properties and pro-inflammatory action. The authors report the case of a 20 year-old girl with cutaneous ulcers comparable with pyoderma gangrenosum, growth failure and chronic anemia, who was given the diagnosis of hyperzincemia and hypercalprotectinemia. Measurement of serum zinc and calprotectin concentrations are indicated in these cases.
Subject(s)
Metal Metabolism, Inborn Errors/pathology , Pyoderma Gangrenosum/pathology , Cyclosporine/therapeutic use , Dermatologic Agents/therapeutic use , Female , Glucocorticoids/therapeutic use , Humans , Leukocyte L1 Antigen Complex/blood , Metal Metabolism, Inborn Errors/drug therapy , Prednisolone/therapeutic use , Treatment Outcome , Young Adult , Zinc/bloodABSTRACT
Abstract Hyperzincemia and hypercalprotectinemia is a rare inflammatory disease caused by a mutation in the PSTPIP1 gene, with a dysregulation of calprotectin metabolism. Calprotectin is a zinc-binding protein with antimicrobial properties and pro-inflammatory action. The authors report the case of a 20 year-old girl with cutaneous ulcers comparable with pyoderma gangrenosum, growth failure and chronic anemia, who was given the diagnosis of hyperzincemia and hypercalprotectinemia. Measurement of serum zinc and calprotectin concentrations are indicated in these cases.