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.
© 2019 Wiley Periodicals, Inc.
MeSH terms
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Anti-Allergic Agents / therapeutic use
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Cetirizine / therapeutic use
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Child, Preschool
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DNA-Binding Proteins / deficiency*
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DNA-Binding Proteins / genetics
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Dermatologic Agents / administration & dosage
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Female
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Humans
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Immunologic Deficiency Syndromes / diagnosis*
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Immunologic Deficiency Syndromes / genetics
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Mometasone Furoate / administration & dosage
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Mutation
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Panniculitis / diagnosis*
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Panniculitis / drug therapy
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Panniculitis / genetics
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Skin / pathology
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Trans-Activators
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Transcription Factors / deficiency*
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Transcription Factors / genetics
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Ubiquitin-Specific Proteases
Substances
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Anti-Allergic Agents
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DNA-Binding Proteins
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Dermatologic Agents
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MYSM1 protein, human
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Trans-Activators
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Transcription Factors
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Mometasone Furoate
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Ubiquitin-Specific Proteases
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Cetirizine