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01.12.2014 | Case report | Ausgabe 1/2014 Open Access

Italian Journal of Pediatrics 1/2014

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

Italian Journal of Pediatrics > Ausgabe 1/2014
Claudia Pasqualini, Mauro Jorini, Ines Carloni, Mirella Giangiacomi, Valentina Cetica, Maurizio Aricò,, Fernando Maria de Benedictis
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1824-7288-40-17) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

CP identified the case, helped in making the diagnosis and worked on bibliography. MJ was involved in the diagnostic process, therapeutic decisions and follow up. IC drafted the manuscript. MG made substantial contribution to analysis and interpretation of immunohistochemical data. VC performed mutation analysis. MA supervised mutation analysis and revised the manuscript. FMdB supervised the diagnostic and therapeutic approach, and critically revised the manuscript. All authors read and approved the final manuscript.


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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