Paraneoplastic Dermatosis in a Patient with Anaplastic Large-Cell Lymphoma: Case Report and Literature Review

Abstract

Background/Aims: Paraneoplastic dermatoses are skin disorders that are associated with malignancy. Anaplastic large T-cell lymphoma (ALTCL) has rarely been associated with paraneoplastic skin manifestations such as gangrenous foot ulcers and erythroderma. Methods: We describe a case of ALTCL presenting as a large annular skin rash. The clinical picture, course, and treatment will be discussed along with current hypotheses on the mechanism of paraneoplastic syndromes. Results: Skin manifestations in ALTCL most commonly arise in two distinct ways; either as primary cutaneous lymphoma manifestation or as systemic disease with secondary metastasis. Less commonly, systemic disease causes skin manifestations secondary to a paraneoplastic process without infiltration of malignant cells. This is thought to be mediated by an immunologic reaction to tumor antigen or the result of cytokines and other inflammatory markers produced by the tumor itself. Conclusion: Paraneoplastic dermatoses could be the initial presentations of systemic lymphoma. Knowledge about their association with anaplastic large-cell lymphoma may help with timely diagnosis. In a patient with unexplained dermatosis associated with B symptoms who is unresponsive to topic treatment, an investigation for systemic lymphoma workup is warranted.

Erythroderma as A Paraneoplastic Cutaneous Disorder in Systemic Lymphoma

“…Less commonly, paraneoplastic erythroderma is associated with acute myeloid leukaemia and solid tumours [7,8]. Paraneoplastic erythroderma associated with systemic lymphoma is exceptionally rare.…”

Metastatic pulmonary melanoma complicated with erythroderma and recurrent sepsis

“…Cutaneous manifestations linked to melanoma are scarce and of unknown underlying mechanism. The diagnosis is of exclusion and should meet the criteria of Curth's postulates [ 5 ]. The best documented dermatoses in relation are dermatomyositis and pemphigus vulgaris [ 6 ].…”

Malignancy‐associated generalised exfoliative dermatitis: A retrospective study in a single‐centre Asian cohort

“…2 Various studies have demonstrated the incidence of this relatively rare disorder; a study in an Indian population documented incidence around 35 per 100,000 dermatology patients, 3 and a study of a Chinese population with incidence around 13 per 100,000 dermatology patients. 4 The causes of erythroderma include exacerbation of existing/underlying dermatoses (such as psoriasis, atopic dermatitis, pemphigus), cutaneous drug eruptions 5 and malignancy-related erythroderma either from paraneoplastic causes (representing particular cutaneous manifestations of an underlying malignancy without infiltration of malignant cells 6 ) or from primary cutaneous malignancies such as mycosis fungoides, Sézary syndrome and adult T-cell leukaemia/lymphomas. 7 Despite malignancy-related erythroderma constituting only a small fraction of erythroderma cases, 8 clinical implications on diagnosis and surveillance can be profound.…”

“…The causes of erythroderma include exacerbation of existing/underlying dermatoses (such as psoriasis, atopic dermatitis, pemphigus), cutaneous drug eruptions 5 and malignancy‐related erythroderma either from paraneoplastic causes (representing particular cutaneous manifestations of an underlying malignancy without infiltration of malignant cells 6 ) or from primary cutaneous malignancies such as mycosis fungoides, Sézary syndrome and adult T‐cell leukaemia/lymphomas 7 …”

“…We herein use the term malignancy‐related erythroderma to encompass paraneoplastic erythroderma (representing particular cutaneous manifestations of an underlying malignancy without infiltration of malignant cells into the skin 6 ) as well as primary cutaneous malignancies such as mycosis fungoides, Sézary syndrome and adult T‐cell leukaemia/lymphomas 7 . We also adopted the definition from the Curth criteria (1976) 9 for the identification of paraneoplastic dermatoses, characterised by the following features: concurrent onset of the dermatosis with the primary neoplasm, parallel development/resolution with primary neoplasm, specificity between tumour type and cutaneous eruption type, and significant statistical and genetic association between dermatosis and neoplasm.…”