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. © 2014 Pasqualini et al.; licensee BioMed Central Ltd.
CITATION STYLE
Pasqualini, C., Jorini, M., Carloni, I., Giangiacomi, M., Cetica, V., Aricò, M., & De Benedictis, F. M. (2014). Cytophagic histiocytic panniculitis, hemophagocytic lymphohistiocytosis and undetermined autoimmune disorder: Reconciling the puzzle. Italian Journal of Pediatrics, 40(1). https://doi.org/10.1186/1824-7288-40-17
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