Title |
Cytophagic histiocytic panniculitis, hemophagocytic lymphohistiocytosis and undetermined autoimmune disorder: reconciling the puzzle
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Published in |
Italian Journal of Pediatrics, February 2014
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DOI | 10.1186/1824-7288-40-17 |
Pubmed ID | |
Authors |
Claudia Pasqualini, Mauro Jorini, Ines Carloni, Mirella Giangiacomi, Valentina Cetica, Maurizio Aricò,, Fernando Maria de Benedictis |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Germany | 1 | 100% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 30 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 5 | 17% |
Student > Postgraduate | 4 | 13% |
Student > Doctoral Student | 3 | 10% |
Student > Master | 3 | 10% |
Other | 2 | 7% |
Other | 7 | 23% |
Unknown | 6 | 20% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 13 | 43% |
Agricultural and Biological Sciences | 5 | 17% |
Immunology and Microbiology | 1 | 3% |
Nursing and Health Professions | 1 | 3% |
Psychology | 1 | 3% |
Other | 1 | 3% |
Unknown | 8 | 27% |