Clonal Cytophagic Histiocytic Panniculitis in Children May Be Cured by Cyclosporine A

Abstract

Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis in childhood, associated either with nonmalignant conditions or with subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and often also associated with macrophage activation syndrome (MAS). Discriminating between these 2 conditions is therapeutically important because nonmalignant CHP often improves under cyclosporine and prednisone, whereas most cases of SPLT may be best treated with more aggressive therapy. We report the cases of a 6-month-old boy and a 16-month-old girl who, after viral infection, developed multiple infiltrating skin nodules on the limbs and face, associated with MAS. Histopathologic findings for skin biopsy specimens revealed CHP associated with heavily cellular lobular panniculitis. Hemophagocytosis and immunohistochemical staining features were consistent with typical characteristics of in situ MAS in adipose tissue: the lymphocytes were mostly TCD8+ cells with an activated phenotype (human leukocyte antigen (HLA) -DR+) and expressed interferon-g; CD68+ macrophages expressed tumor necrosis factor-a and interleukin-6. A monoclonal rearrangement of the T-cell receptor g gene was present in skin tissue but not in peripheral blood or bone marrow lymphocytes. Cyclosporine A treatment resulted in the complete remission of cutaneous and systemic manifestations in both patients for 66 and 29 months, respectively. This report suggests that the diagnosis of a reactive T-cell lymphoproliferation should be the treatment of choice in young children with severe CHP, even if there is a SPTCL-like aspect with an in situ T-cell clonality. It also suggests that CSA is the optimal treatment of this condition and postulates the possible pathologic process underlying this efficacy. Pediatrics 2013;132:e545-e549

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

“…This approach would have a beneficial effect on treatment planning towards oncological rather than anti-inflammatory therapy. Bader-Meunier et al [21] recently emphasized that HLH-associated CHP may be diagnosed despite monoclonal T-cell proliferation that mimics SPTL and is best treated by prednisone and cyclosporine A, at least in children. It has been suggested that this florid clonal T-cell proliferation is reactive, probably driven by a strong immune reaction against EBV infection [22].…”

Bilateral ptosis as first presentation of cytophagic histiocytic panniculitis: a case report

“…Most importantly, the histological findings were different from those of lupus panniculitis: characteristic phagocytic histiocytes (“bean bag” cells) and coagulative necrosis were showed without focal calcifications or cysts bounded by amorphic eosinophilic material. Thus, lupus panniculitis could be excluded [10–12]. …”

Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone

“…[13] The presence of MAS or hemophagocytosis (HPS) might change the management approach and outcome in SPTCL. [14] One study reported 5-year overall survival rates of 91% and 46% in SPTCL patients without and with an HPS, respectively.…”