Aggressive CD30 Large Cell Lymphoma after Cyclosporine Given for Putative Atopic Dermatitis

Abstract

Atopic dermatitis (AD) is not regarded as a predisposing condition for cutaneous T cell lymphoma. Cyclosporine (CsA) is an efficient therapy in AD, and its side effects, including lymphocytic proliferation, are rare at the low dose used in such cases. So far 3 cases of patients who developed cutaneous T cell lymphoma under CsA treatment for atopy have been described. An adult patient with a history of AD received CsA therapy because of an atypical flare-up of his eruption. He rapidly developed papular atypical lesions, then followed by the onset of a voluminous cutaneous tumor leading to the diagnosis of mycosis fungoides (MF) transformed in CD30+ large cell lymphoma with nodal and blood involvement. No improvement occurred after withdrawing of CsA, and he required a polychemotherapy and an allograft. This case illustrates the dramatic evolution of an MF under CsA given in the hypothesis of AD. It stresses the fact that skin biopsies should be taken in case of atypical AD to rule out MF and avoid such an aggressive evolution under immunosuppressive therapy.

Primary cutaneous CD30+ lymphoproliferative disorder in an atopic dermatitis patient on cyclosporine therapy

“…CD30+ lymphoproliferative disorder in AD patients during cyclosporine therapy is rare and only four cases have been described [ Table 1]. [1][2][3][4] Cyclosporine is also used for psoriasis, which is accompanied by similar lymphoproliferative disorder. While some reports suggest no significant relation between AD and lymphoma, others suggest the positive correlation between cutaneous lymphoproliferative disorder and AD.…”

“…However, after using cyclosporine for AD, primary cutaneous CD30+ lymphoproliferative disorder (including lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (ALCL), and borderline lesion) is sporadically observed. [1][2][3][4] Recent report indicated that CD30+ T lymphocytes are up-regulated in the lesional skin of AD by mast cells which show the regulatory roles on both innate and acquired immunities. [5] A 57-years-old man was treated by topical corticosteroid ointment and antihistamine by a local dermatologist because of itchy chronic eruptions under a diagnosis of AD for about 20 years.…”

Mycosis Fungoides in the Setting of T-cell Large Granular Lymphocyte Proliferative Disorder

“…Speculative, is it, in our case, a common stem cell/lymphoid precursor, having undergone one or more somatic events predisposing to cytokine-independent activation ofthe Jak3/Stat3 pathway, generated, through divergent differentiation, two distinct aberrant populations of T lymphocytes (i.e., CD8L GL and CD4'^ cells, respectively). Secondly, loss of immune competence resulting from long-standing CyA treatment may have favoured development and/or progression of MF in our case (4,11): cases have been reported where treatment with low-dose CyA (<5 mg/kg/day) following a misdiagnosis of inflammatory dermatoses led to clinical worsening and aggressive transformation of MF (16,17). Of note, in our patient the effect of continuous CyA administration may have been compounded by the relative immunosuppression which is known to be inherent in patients with lymphoproliferative conditions, including T-LGL disorders.…”

Cutaneous T Cell Lymphoma Complicating Severe Atopic Dermatitis

“…Most of them were CD30+ cutaneous lymphomas (6 cases), including lymphomatoid papulosis (2 cases) [9,10,11,12,13], but other types of CTCL have also been reported in association with AD: Sézary syndrome [14, 15] and mycosis fungoides [16, 17]. …”

“…Six of the 8 cases of CD30+ CTCL associated with severe longstanding AD occurred in the context of a ciclosporin treatment [9,10,11,12,13]. Concerns have recently been raised regarding a possible association between use of topical calcineurin inhibitors and cutaneous lymphoma [19].…”