Epidermotropic CXCR3 positive marginal zone lymphoma: a distinctive clinical histopathological entity potentially originating in the skin; it does not always indicate splenic marginal zone lymphoma

Primary cutaneous marginal zone lymphoma with unusual histopathological features: Subcutaneous involvement and epidermotropism with massive fibrosis

“…Our patient's nail abnormalities did not improve neither with methotrexate 5 or TNF-alpha inhibitors, 6 which are mainly Th1-mediated lymphocytic response inhibitors, nor with cyclosporine, 7 a non-selective lymphocytic inhibitor. 5 The relevant improvement in nail manifestations of our patient after treatment with dupilumab, a specific Th2 pathway blocker, 8 could indicate the presence of an inflammatory infiltrate at the nail matrix or the nail bed formed predominantly by Th2 lymphocytes, similar to what we currently know in atopic eczema. Moreover, it does not seem that the polarisation of na€ ıve T lymphocytes to Th1 and Th17 after treatment with Th2 inhibitors can trigger psoriatic onychopathy in patients with atopic dermatitis; however, future observations and studies will be necessary to know this detail with a greater accuracy.Dupilumab can improve nail manifestations associated with atopic dermatitis, may be acting in a similar way to that observed on the skin.…”

“…4 However, a case of epidermotropic CMZL without splenic involvement has been reported as well. 5 Although our second patient showed no evidence of splenic or bone marrow involvement, patients with epidermotropic CMZL should be examined thoroughly to rule out the systemic involvement of MZL.…”

“…4 Epidermotropism is attributed to the local intraepidermal milieu attracting neoplastic immune cells. 4,5 It has been postulated that interferon-c-induced protein 10, expressed on the keratinocytes and stromal cells of the spleen and bone marrow, attracts neoplastic cells of CMZL with aberrant expression of CXCR3. Therefore, the risk of involvement of the spleen and bone marrow may increase with epidermotropic CMZL.…”

“…Epidermotropism has been reported to be associated with several diseases including mycosis fungoides, Sézary syndrome, and Langerhans cell histiocytosis 4 . Epidermotropism is attributed to the local intraepidermal milieu attracting neoplastic immune cells 4,5 . It has been postulated that interferon‐γ‐induced protein 10, expressed on the keratinocytes and stromal cells of the spleen and bone marrow, attracts neoplastic cells of CMZL with aberrant expression of CXCR3.…”

Epidermotropic B-Cell Lymphoma: A Case of Secondary Cutaneous Splenic Marginal Zone B-Cell Lymphoma With Autoinvolutive and Recurrent Cutaneous Lesions

“…[10][11][12][13][14][15][16][17] Other cases have been diagnosed as primary epidermotropic cutaneous MZL because no evidence of clear extranodal MZL has been documented. [18][19][20][21][22] However, some patients had bone marrow involvement or splenomegaly at presentation or disease evolution. Table 1 shows the clinical features of cutaneous MZL cases with epidermotropism.…”