Extensive pyoderma gangrenosum-like lesions revealing a case of hyperzincemia and hypercalprotectinemia: when to suspect it?

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.

Diagnostic potential of histochemical demonstration for copper-orcein stain.

The histochemical demonstration of hepatic copper is important in the diagnosis of paediatric copper storage disorders. Conflicting results have been published regarding ability of different histochemical stains to demonstrate copper storage in the liver. Hence the authors retrospectively analysed eighty-two liver biopsies from 82 patients of Indian childhood cirrhosis (ICC), 59 males and 23 females aged between 1-4 years (mean age 3.1 years). All cases were stained with orcein stain. On the basis of histological picture the liver biopsies were divided into the three histological grades I, II and III. Orcein positively was graded from I to IV. All cases showed positivity with orcein stain. Most cases showed grades II and III of orcein positivity. The association between histological and orcein grades was significant. The present study demonstrates the diagnostic utility of orcein stain in liver copper storage disorder, Indian childhood cirrhosis. Variable copper content in the same histological grade of the disease could be due to individual factors such as genetic milieu which determine the amount of copper liver can store without toxicity.