Neutrophilic Panniculitis in a child with MYSM1 deficiency

Pediatr Dermatol. 2019 Mar;36(2):258-259. doi: 10.1111/pde.13757. Epub 2019 Feb 12.

Abstract

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.

Keywords: MYSM1 deficiency; myelodysplasia; neutrophilic panniculitis; primary immunodeficiency.

Publication types

  • Case Reports

MeSH terms

  • Anti-Allergic Agents / therapeutic use
  • Cetirizine / therapeutic use
  • Child, Preschool
  • DNA-Binding Proteins / deficiency*
  • DNA-Binding Proteins / genetics
  • Dermatologic Agents / administration & dosage
  • Female
  • Humans
  • Immunologic Deficiency Syndromes / diagnosis*
  • Immunologic Deficiency Syndromes / genetics
  • Mometasone Furoate / administration & dosage
  • Mutation
  • Panniculitis / diagnosis*
  • Panniculitis / drug therapy
  • Panniculitis / genetics
  • Skin / pathology
  • Trans-Activators
  • Transcription Factors / deficiency*
  • Transcription Factors / genetics
  • Ubiquitin-Specific Proteases

Substances

  • Anti-Allergic Agents
  • DNA-Binding Proteins
  • Dermatologic Agents
  • MYSM1 protein, human
  • Trans-Activators
  • Transcription Factors
  • Mometasone Furoate
  • Ubiquitin-Specific Proteases
  • Cetirizine