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Skin diseases of the elderly
Published in Robert A. Norman, Geriatric Dermatology, 2020
Cutaneous T cell lymphoma (CTCL) includes a constellation of diseases of malignant clonal T lymphocytes which present initially on the skin. Mycosis fungoides and Sézary syndrome are the most common and their malignant cells are mature post-thymic T cells, usually helper/inducer (CD4) phenotypes. Mycosis fungoides (MF) (Figure 30) most often affects male adults in their 5th decade and has a predilection for African-Americans. The lesions classically progress slowly over a period of 5 to 10 years through the defined cutaneous phases: erythematous scaly patches that may be poikilodermic, plaques and tumors (nodular and ulcerated). As MF advances, the chances of extra-cutaneous involvement increase. The most common sites are the regional lymph nodes, the lungs, liver, spleen, gastrointestinal tract and brain.
Lymphoma
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
These are often low grade (lymphoma cutis) and require only gentle local treatment from time to time, but more extensive high-grade lymphoma can develop, as shown in Figure 16.6. Mycosis fungoides is a characteristic T-cell skin lymphoma, which has a long pretumour phase before developing into the characteristic skin infiltration, which can be widespread. It responds poorly to chemotherapy. Less severe forms can respond to PUVA (psoralens and ultraviolet A exposure) and for others local irradiation is required. In widespread disease, the entire body can be affected when irradiation of the whole body with electrons will be required.
The lymphoreticular system and bone marrow
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
This tumour presents as a skin rash (see Chapter 19), initially with patches and plaques and later with nodules. Histologically, there is infiltration of the dermis and epidermis by CD4+ T cells. It can be difficult to make this diagnosis in its early stages because it can resemble dermatitis. Mycosis fungoides tends to be indolent in its behaviour, at least initially. A minority of patients develop high-grade transformation and die as a consequence.
Long-term effects of total skin electron beam therapy for mycosis fungoides on hair and nail loss and regrowth
Published in Journal of Dermatological Treatment, 2022
Debra L. Breneman, Alyssa Breneman, Elaine Ballman, John C. Breneman
Mycosis fungoides (MF) is a mature T-cell non-Hodgkin lymphoma characterized by skin involvement. It is the most common of cutaneous lymphomas. Clinically, this disease most often manifests as patches, plaques, and tumors (1). Histopathologically, it is characterized by superficial infiltrates of atypical epidermotropic T cells (2). Treatments for MF are varied, and the choice is largely based on the stage of the disease (3). Therapies for early-stage MF (IA–IIA) are largely skin-directed, including topical corticosteroids, chlormethine or retinoids, phototherapy, and radiotherapy. Systemic treatments are often used for more advanced disease, with or without concomitant skin-directed therapies (3). Total skin electron beam therapy (TSEBT) has been proven to be one of the most effective treatments for MF in all stages, with lower clinical response rates as the disease progresses (3).
Overall survival and registration of cutaneous T-cell lymphoma patients in Sweden: a multi-center cohort and validation study
Published in Acta Oncologica, 2022
Julia Nenonen, Anna H. Winther, Ellinor Leijonhufvud, Emma Belfrage, Karin E. Smedby, Hanna Brauner
Primary cutaneous T-cell Lymphoma (CTCL) is a group of rare non-Hodgkin lymphomas characterized by malignant T cells that are initially only present in the skin. Mycosis fungoides (MFs) represent the most common primary CTCL accounting for nearly 60% of all cases with an incidence of 0.2–0.6 cases/100,000 person years in Europe and the USA [1–4]. MF often has an indolent clinical course characterized by superficial skin patches and/or elevated plaques. However, for unknown reasons some patients develop a more aggressive form of the disease with skin tumors, erythroderma, and/or extracutaneous involvement [5]. Consequently, the prognosis of MF is often favorable, but may still vary considerably among patients and is dependent on the stage of the disease [6]. Early-stage disease (stage IA–IIA) generally has a favorable prognosis with a 5-year disease-specific survival of 89%–98%, while advanced-stage MF (IIB-IV) has a worse prognosis with a 5-year disease-specific survival reported to range between 18 and 67% [7,8]. Population-based data on the overall survival (OS) among CTCL patients are mostly limited to studies that are small, old, or with short follow-up considering the often indolent course of CTCL [9–11].
Lymphoprolipherative skin reactions induced by anti-TNFα: an open question
Published in Journal of Dermatological Treatment, 2020
Vasiliki Nikolaou, Maria Gerochristou, Leonidas Marinos, Afroditi Economidi, Dimitra Voudouri, Dimitris Rigopoulos, Alexander J Stratigos
Five anti-TNFα treated patients with skin lesions, clinically suspicious for Mycosis Fungoides (MF), were referred to our Lymphoma Clinic. There were four males and one female, aged between 25 and 63 years old. Ankylosing spondylitis was the most prevalent diagnosis (3 patients), while one suffered from psoriatic arthritis and another one from Crohn’s disease. Adalimubab was given in three cases, Infliximab in one and both agents in one patient. Clinical presentation varied from erythrodermia to typical follicular MF lesions. Drug discontinuation led to complete resolution of the skin lesions in three cases, while a very short course of phototherapy was effective in two patients. However, in two of them, late relapse of the rash was noticed and the diagnosis of mycosis fungoides was established. Table 1 presents the clinical characteristics and follow up of all five patients. Histological immunochemistry and molecular analysis are summarized in Table 2. Figure 1 represents one case of a FMF-like reaction that finally progressed to true MF, whereas Figure 2 presents a case that has been considered pseudolumphomatoid FMF like reaction.