![Sample our Medicine, Dentistry, Nursing & Allied Health journals, sign in here to start your FREE access for 14 days]({"type":"image" , "src" : "/sda/5944/TOC-Subject-Sample-2021-Medicine-Dentistry-Nursing-Allied-Health.jpg"})
Abstract
Erythroderma describes complete or near-complete skin surface erythema of any cause. Cutaneous T-cell lymphoma accounts for a minority of cases of erythroderma and consists of erythrodermic mycosis fungoides, Sézary syndrome. Both adult T-cell leukemia/lymphoma and T-cell prolymphocytic leukemia can also rarely present with erythroderma. Diagnosis may be extremely challenging because benign disorders may have overlapping features with those of lymphoma. Prognosis is poor with median survival of approximately 2 years. The evidence base for therapeutic approaches relies on cohorts and case series and more recently Phase II trials. Improved patient selection and identification of appropriate conditioning regimens for reduced intensity allogeneic hematopoetic transplant are likely to improve survival, although a significant number of patients may not be fit for transplant because of advanced age and comorbidities.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the article. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
Cutaneous T-cell lymphoma (CTCL) may present with erythroderma.
In the context of erythroderma, no single feature is diagnostic of CTCL. Suggestive clinical features include fever, edema, leonine facies and palmoplantar keratoderma. A comprehensive diagnostic workup should include peripheral blood for FBC, lymphocyte count, lymphocyte subsets, eosinophil count, lactate dehydrogenase, β2 microglobulin, human T-lymphotropic virus type 1, Sézary cell count, electrolytes with optional fluorescence-activated cell sorting analysis using antibodies for the V-β region. Immunohistochemistry of skin biopsy should as a minimum include CD4, CD8, CD2, CD3, CD7, CD30. T-cell receptor gene analysis should be undertaken on blood, skin and any other tissues sampled and results compared to establish that clones are identical to the skin tumor clone.
Classification and staging of mycosis fungoides and Sézary syndrome are based on the tumor, lymph node, visceral, blood system developed and revised for CTCL by the EORTC/ISCL and adopted by the American Joint Committee on Cancer.
The severity-weighted assessment tool or modified severity-weighted assessment tool is recommended for the assessment of skin disease, and a global disease scoring system captures a composite disease assessment score based on all potential tumor compartments including skin, blood and node.
In a retrospective analysis, patients with erythrodermic CTCL (T4) at diagnosis had the worst overall survival of any skin stage (median 3.9 years), and overall survival is worse (2.4 years) in those patients presenting with skin tumors associated with erythroderma.
The evidence base for the treatment of eCTCL is mainly based on uncontrolled cohort or Phase II nonrandomized studies. In all patients, supportive care is particularly important to maintain homeostasis, reduce risks of secondary skin infection and control skin-specific symptoms of pruritus, pain and burning.
Treatment options for patients with stage IIIA disease include methotrexate, extracorporeal photopheresis/interferon/bexarotene combinations, biologics such as histone deacetylase inhibitors (licensed in the USA) and clinical trials.
Treatment options for patients with stage IIIB/stage IVA1 disease include extracorporeal photopheresis/interferon/bexarotene combinations, biologics such as histone deacetylase inhibitors (licensed in the US), alemtuzumab and clinical trials.
Treatment options for patients with stage IVA2/IVB disease include alemtuzumab, biologics such as histone deacetylase inhibitors (licensed in the US), radiotherapy (including total skin electron beam therapy) chemotherapy and clinical trials.
Chemotherapy can provide useful palliation in erythrodermic mycosis fungoides/Sézary syndrome. Although good overall responses are reported with both single agent and combination regimens, response duration is short. In view of the similar efficacy and lower toxicity, single-agent chemotherapy is preferable as palliative therapy for patients with erythrodermic CTCL.
Nonmyeloablative stem cell transplantation is an important consideration for those patients with a good performance status who have achieved a reasonable partial or complete response. Although the best conditioning regimen has yet to be established, T-cell depletion is associated with early disease relapse and should be avoided.