PLAID syndrome: Characteristic presentation and a novel therapeutic option

James Shea*, Thy Huynh, Joshua Milner, Sarah Chamlin

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

PLCG2-associated antibody deficiency and immune dysregulation (PLAID) is an autosomal dominant inherited disease caused by genomic deletion in PLCG2 and is characterized by cold urticaria, humoral immune deficiency, cutaneous granulomas, and autoimmune disease. The patient described in this case had a typical presentation for a PLAID phenocopy and experienced intense pruritus, a common complication of PLAID, starting in early childhood. After trialing H1 and H2 blockers with no improvement, oral glycopyrrolate was used with near resolution of the patient's symptoms. Given that the pruritus in PLAID is related to sweat-induced evaporative cooling, practitioners who encounter this disease should consider glycopyrrolate in their management of PLAID-associated pruritus.

Original languageEnglish (US)
Pages (from-to)147-149
Number of pages3
JournalPediatric dermatology
Volume37
Issue number1
DOIs
StatePublished - Jan 1 2020
Externally publishedYes

Keywords

  • PLAID
  • pruritus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Dermatology

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