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Open Access 01-12-2014 | Case report

Cytophagic histiocytic panniculitis, hemophagocytic lymphohistiocytosis and undetermined autoimmune disorder: reconciling the puzzle

Authors: Claudia Pasqualini, Mauro Jorini, Ines Carloni, Mirella Giangiacomi, Valentina Cetica, Maurizio Aricò,, Fernando Maria de Benedictis

Published in: Italian Journal of Pediatrics | Issue 1/2014

Abstract

Cytophagic histiocytic panniculitis is a rare disease, associated with either nonmalignant conditions or subcutaneous panniculitis-like T-cell lymphoma, and often also associated with hemophagocytic lymphohistiocytosis (HLH). We report the case of a 11-year-old boy with a history of secondary HLH who, after a local trauma, developed a painful, indurated plaque over the right thigh associated with relapsing HLH. Histopathologic findings from skin biopsy specimens revealed significant lobular panniculitis with benign histiocytes showing hemophagocytosis. High-dose intravenous methylprednisolone and cyclosporine A treatment was highly effective. A genetic study after a new, relapsing episode of HLH revealed an heterozygous missense mutation on STX 11 gene inherited from the mother.

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Title: Cytophagic histiocytic panniculitis, hemophagocytic lymphohistiocytosis and undetermined autoimmune disorder: reconciling the puzzle
Authors: Claudia Pasqualini
Mauro Jorini
Ines Carloni
Mirella Giangiacomi
Valentina Cetica
Maurizio Aricò,
Fernando Maria de Benedictis
Publication date: 01-12-2014
Publisher: BioMed Central
Published in: Italian Journal of Pediatrics / Issue 1/2014
Electronic ISSN: 1824-7288
DOI: https://doi.org/10.1186/1824-7288-40-17

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Cytophagic histiocytic panniculitis, hemophagocytic lymphohistiocytosis and undetermined autoimmune disorder: reconciling the puzzle

Abstract

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

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