Neurolymphomatosis in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma mimicking Hansen's disease

Abstract

Neurolymphomatosis (NL) refers to nerve infiltration by neurotropic neoplastic cells in the setting of a known or an unknown hematological malignancy. It typically presents as painful or painless peripheral mononeuropathy, mononeuritis multiplex, polyneuropathy, polyradiculopathy, or cranial neuropathy. A 32-year-old male presented with a hyperpigmented hypoesthetic plaque over the anterolateral aspect of the right leg with thickening of the right common peroneal nerve and foot drop clinically diagnosed as Hansen's disease. Biopsy taken from skin showed infiltrates of pleomorphic small and medium sized lymphocytes in the dermis and subcutis. On immunohistochemistry, the cells were positive for CD3, CD4 and negative for CD8, CD20, and CD30. Ultrasonography-guided fine-needle aspiration of the thickened nerve showed infiltrates of atypical lymphoid cells. Based on these findings, a diagnosis of NL in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma was made. The disease responded to systemic chemotherapy and localized radiotherapy with no evidence of relapse during 3 years follow-up. NL in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma presenting with manifestations redolent of Hansen's disease is not described in available literature. This case also demonstrates the utility of fine needle aspiration of nerve, a minimally invasive procedure in the diagnosis of NL.

Putative mechanisms for spread and transformation of cutaneous T‐cell lymphoma to neurolymphomatosis

“…Several cases of NL arising in patients with T‐cell lymphoma have been reported, but none have demonstrated direct epineural invasion by cells in the subcutaneous tissues, despite disease present in subcutaneous tissues overlying a relatively superficial nerve . In a case reported by Hanna et al, the patient described developed high ulnar neuropathy, as well as median neuropathies localized to the wrists, in the setting of multiple skin plaques of the upper extremities.…”

“…Mycosis fungoides is an erythrodermic variant of T‐cell lymphoma, progressing over years with cutaneous plaques, patches, and nodules caused by clonal T cells invading the skin . Spread to the nervous system is rare (2%‐14% of cases), but most commonly manifests with spread to the leptomeninges, and less often as neurolymphomatous involvement of the peripheral nerves …”

Primary cutaneous CD4+ small/medium pleomorphic T‐cell lymphoproliferative disorder: Where do we stand? A systematic review

“…A few unusual clinical presentations have been reported such as acneiform , alopecia and leprosy‐like disease . Drug‐induced PCSMP‐TLPD has also been reported for the methotrexate‐etanercept combination , vemurafenib , interleukin (IL)‐2 therapy for metastatic melanoma and standard triple‐agent immunosuppressive therapy (cyclosporine, prednisolone, and azathioprine) following heart transplantation surgery , although this is an extremely rare presentation.…”

“…The dermal infiltrate may have a nodular or band‐like distribution with a grenz zone separating it from the epidermis, but focal epidermotropism or extension to the subcutaneous fat may also occur (Figure ). Granulomatous , folliculotropic and neurolymphomatosis patterns have been reported in some cases.…”

Primär kutane CD4+ klein‐ bis mittelgroßzellige pleomorphe T‐Zell‐Lymphoproliferation: Wo stehen wir? Eine systematische Übersicht

“…Es wurde auch über einige wenige atypische klinische Manifestationen berichtet, wie Akne‐ oder Lepra‐ähnliche Veränderungen und Alopezie [–]. Des Weiteren wurde nach Behandlung mit der Kombination Methotrexat‐Etanercept , mit Vemurafenib , Interleukin (IL)‐2 bei metastasiertem Melanom und immunsuppressiver Dreifachstandardtherapie (Cyclosporin, Prednisolon und Azathioprin) nach Herztransplantation über arzneimittelinduzierte PCSMP‐TLPD berichtet, wobei dies jedoch äußerst selten ist.…”