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Primary cutaneous lymphomas are a heterogeneous group of rare lymphoproliferative diseases that are initially limited to the skin but potentially also involve the lymph nodes, blood, and occasionally visceral organs. They develop from mature T cells, B cells, and NK cells. The classification includes a wide range of pathologies, each characterized by distinct clinical symptoms and variations in disease course. Cutaneous lymphomas are typically chronic in nature, and a complete cure is rarely achievable. Since primary cutaneous lymphomas are uncommon and present in many variants, they pose diagnostic and therapeutic challenges that require multidisciplinary collaboration between dermatologists, pathologists, hematologists, and oncologists. We present revised guidelines for the diagnosis and treatment of primary cutaneous lymphomas, with the goal of simplifying the diagnostic and therapeutic procedure in routine clinical practice. The guidelines cover the clinical, histopathological, and dermoscopic characteristics of skin lesions, along with recommendations for the diagnostic and therapeutic management of primary cutaneous lymphomas. When selecting the most suitable therapeutic approach for individual patients diagnosed with primary cutaneous lymphoma, the main factor to consider should be the clinical stage of the disease. However, other factors, such as the patient’s age, overall health, and medical history, should also be taken into account. The choice of therapy should be guided by finding optimal efficacy and safety. The guidelines also include globally recognized therapeutic approaches that are currently not accessible in Poland. The recommendations rely on the existing Polish and global guidelines, and the knowledge and experience of experts.

Primary cutaneous lymphomas are a heterogeneous group of neoplastic diseases in which dermatological examination plays the pivotal role toward a management which must be multidisciplinary by definition [...].

Eyelids may be affected in systemic, ocular adnexal and primary cutaneous lymphomas (PCLs). The frequency of eyelid involvement in PCLs is still not well known and it is not a predilection site for any type. While primary cutaneous T‐cell lymphomas (CTCLs) are more commonly seen than primary cutaneous B‐cell lymphomas (CBCLs), especially mycosis fungoides (MF) as by far the most frequent type, B cell lymphomas are reported to be the commonest type in eyelid localization on the contrary. PCLs may be located on the eyelids, as the sole manifestation or in association with the involvement of other parts of the eye and elsewhere of the body. MF may present with a rich spectrum of clinical features on the eyelids mostly seen in folliculotropic subtype and advanced‐stage disease. Erythematous scaly patches or plaques representing the most commonly encountered eyelid MF lesions may mimic many other dermatological conditions. Diffuse thickening, oedema, poikilodermic changes, atrophy and wrinkling are other suggestive findings of eyelid MF. Milia‐like papules, madarosis and ectropion are also seen in the folliculotropic variant of MF, as ectropion is more typical for Sezary syndrome. Eyelids are also a typical location for tumoural MF which has been suggested as a poor prognostic indicator in MF. Papulonodular lesions, large tumours, ulceration, diffuse infiltration, oedema and subcutaneous atrophy on the eyelids may also be seen in other types of PCLs. Keep in mind, the rich clinical spectrum of PCLs on the eyelids may be crucial in early diagnosis in this special localization. [ABSTRACT FROM AUTHOR]

With this work, we aimed to review the principal benign and malignant tumors (including vascular, keratinocytic/epidermal, melanocytic, hematopoietic, and lymphoid origin), primarily affecting the leg’s skin. The lesions’ location can also help focus on a spectrum of differential diagnoses in clinical practice. All the diseases present the same clinical presentation characterized by erythematous to violaceous nodules. Despite the same clinical presentation, each disease’s prognostic outcome and therapeutic management can be somewhat different. Since clinical diagnosis may sometimes be challenging, histology and immunohistochemistry play a fundamental role in recognizing and staging these types of lesions. Molecular studies can help to determine the exact nature of lesions with no specific characteristics. Kaposi’s sarcoma is an angioproliferative neoplasm that typically occurs in the lower limbs and can enter into differential diagnosis with several other rarer skin diseases. The principal differential diagnosis concerns primary cutaneous lymphomas, of which mycosis fungoides represent the most frequent primary cutaneous T-cell lymphoma. Other rare forms include primary cutaneous B-cell lymphomas, which can be divided into indolent and aggressive forms, such as the primary cutaneous diffuse large B-cell lymphoma, leg type, and lymphomatoid papulomatosis (LyP). In the case of indolent lesions, skin-directed therapies, limited-field radiotherapy, and surgical approaches can be good options. At the same time, different management, with systemic chemotherapy and allogenic bone marrow transplant, is required with aggressive neoplasms, such as blastic plasmacytoid dendritic cell neoplasia or advanced mycosis fungoides. The dermatologist’s role can be crucial in recognizing such diseases and avoiding misdiagnosis, giving the pathologist the correct clinical information for an accurate diagnosis, and starting the suitable therapy.

Primary cutaneous lymphomas (PCLs) are non-Hodgkin's lymphomas present in the skin, with no evidence of extracutaneous disease at the time of diagnosis. PCL is uncommon in children, and those that do exist are mostly of the T-cell lineage. PCL of the B-cell linage is most common in the sixth decade of life and is extremely rare in childhood.

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Previous studies have reported that primary cutaneous lymphomas profoundly influence patients’ health-related quality of life (HRQoL). However, assessment of this psycho-social concept is not common in routine patient care unless required within clinical trials. The aim of this review is to provide a comprehensive overview of HRQoL measures and outcomes in cutaneous lym­phomas in order to inform clinicians. Advanced-stage cutaneous lymphomas were found to be associated with worse HRQoL than early-stage disease. Specifically, progression of the disease, age, sex, psychosocial issues, educational level and therapy were related to the extent of impairment of HRQoL. Treatment response was linked to improved HRQoL, but notably ameliorated HRQoL scores were also reported despite objective disease response. However, the variety of instruments applied to measure HRQoL in cutaneous lymphomas makes it difficult to compare data directly. In conclusion, speciality-specific HRQoL instruments were superior to generic ones, which probably failed to recognize small, but relevant, changes, demonstrating the need for a disease-specific tool.

The Coronavirus disease 2019 (COVID‐19) pandemic spreads quickly all over the world. There are no sufficient data in the literature about COVID‐19 infection and cutaneous lymphomas. This review sheds the light on what is known so far about COVID‐19 with a cutaneous lymphoma perspective. Cutaneous T‐cell lymphoma (CTCL) diagnosis does not represent a predisposing factor to viral infections and most of CTCL patients have indolent disease. However, physicians should be cautious with patients with aggressive primary cutaneous lymphomas and advanced CTCL. Different treatment strategies for cutaneous lymphomas should be taken into consideration during the COVID‐19 pandemic. Thus, it is highly needed to estimate the benefit‐to‐risk ratio on a case‐by‐case basis. [ABSTRACT FROM AUTHOR]

Keywords: primary cutaneous lymphomas; aggressive cutaneous B cell lymphomas; rituximab; target therapy; new monoclonal antibodies Rituximab monotherapy was proven effective in primary cutaneous low-grade B cell lymphomas, whereas the ESMO clinical practice guidelines indicate immunochemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) as first-line treatment for PCDLBCL-LT ([10]). 5 Pham-Ledard, A, Prochazkova-Carlotti, M, Andrique, L, Cappellen, D, Vergier, B, Martinez, F, Multiple genetic alterations in primary cutaneous large B-cell lymphoma, leg type support a common lymphomagenesis with activated B-cell-like diffuse large B-cell lymphoma. Primary cutaneous lymphomas, aggressive cutaneous B cell lymphomas, rituximab, target therapy, new monoclonal antibodies. [Extracted from the article]

Background: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. Aims: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts. Materials and Methods: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/EORTC and 2016-WHO classifications. Results: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein–Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety. Conclusions: Most non-MF/SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction.

Previous studies have reported that primary cutaneous lymphomas profoundly influence patients' health-related quality of life (HRQoL). However, assessment of this psycho-social concept is not common in routine patient care unless required within clinical trials. The aim of this review is to provide a comprehensive overview of HRQoL measures and outcomes in cutaneous lymphomas in order to inform clinicians. Advanced-stage cutaneous lymphomas were found to be associated with worse HRQoL than early-stage disease. Specifically, progression of the disease, age, sex, psychosocial issues, educational level and therapy were related to the extent of impairment of HRQoL. Treatment response was linked to improved HRQoL, but notably ameliorated HRQoL scores were also reported despite objective disease response. However, the variety of instruments applied to measure HRQoL in cutaneous lymphomas makes it difficult to compare data directly. In conclusion, speciality-specific HRQoL instruments were superior to generic ones, which probably failed to recognize small, but relevant, changes, demonstrating the need for a disease-specific tool. [ABSTRACT FROM AUTHOR]

Background: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. Aims: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts. Materials and Methods: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/ EORTC and 2016-WHO classifications. Results: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein-Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety. Conclusions: Most non-MF/ SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction. [ABSTRACT FROM AUTHOR]

The primary cutaneous lymphomas are a heterogeneous group of T-, Natural Killer- and B- cell neoplasms with a wide range of clinical and pathological presentations, and with very different prognoses compared to systemic lymphomas. Recent studies have shown that the skin microenvironment, which is composed of various immune cell subsets as well as their spatial distribution and T-cell interactions through different chemokines and cytokines, has an important role in the development and pathogenesis of cutaneous lymphomas and has assisted in the development of novel and more effective immunotherapies. The following review will focus on the major subtypes of primary cutaneous lymphomas, including the clinical and histological patterns, molecular hallmarks, and current and future treatment strategies. [ABSTRACT FROM AUTHOR]

Objectives: The therapy of advanced, relapsed or refractory primary cutaneous lymphomas is often unsatisfactory. Recent data indicate a favourable pharmacokynetic, pharmacodynamic and toxicity profile of pegylated liposomal doxorubicin (Peg-Doxo) in primary cutaneous T-cell lymphomas, while in primary cutaneous B-cell lymphomas (PCBCLs), the drug efficacy has never been assessed so far. Methods: We performed a prospective phase II pilot clinical trial of Peg-Doxo monotherapy (20 mg/m2) in PCBCLs. One patient had a marginal zone B-cell lymphoma and four were affected by diffuse large B-cell lymphoma-leg type, all with widespread nodular lesions. Results: All the patients achieved a complete response (CR = 100%) in a short period of time (median 3 months), even when pretreated with radio-chemotherapy. Two experienced a relapse. At follow-up, one patient died for progressive disease; four are in CR after 5, 52, 63 and 69 months. As concerning the toxicity profile, the treatment was well-tolerated, no one decreased or delayed the dose. The haematological toxicity was mild with only one case of grade III neutropenia; a patient showed a grade I neurotoxicity. Dermatological toxicity, in particular the palmar–plantar erythrodysesthesia, did not occurred, probably because of both the low dosages of Peg-Doxo monotherapy and the oral prophylaxis with pyridoxine. Conclusions: In spite of the small number of patients, it emerges that monochemotherapy with Peg-Doxo has a significantly high clinical activity and a good safety profile in PCBCLs, even in aggressive forms, compared with other therapeutic regimens, which are completely reviewed. It suggests the need of further investigations in this field. [ABSTRACT FROM AUTHOR]

The cutaneous environment hosts a number of hematopoietic neoplasms that are dominated by primary cutaneous (PC) T-cell lymphomas. Recent progress in molecular biology and immunology has provided tools to investigate the pathogenesis and the biology of these neoplasms. This review highlights newest findings concerning the immune biology of CD4+CD56+ hematodermic neoplasms, and PC T-cell and B-cell lymphomas, speculating how these can be translated into more sophisticated, biology-based treatment approaches in the near future. [ABSTRACT FROM AUTHOR]

Primary cutaneous lymphomas (PCLs) consist of an heterogenous group of rare malignancies. Cutaneous T-cell lymphomas are the most common among them, with mycosis fungoides being the main representative. The etiology of PCLs is rather unknown; however, there are indications that genetic factors may play a role in CTCL developments [1]. Several studies have examined the incidence of second malignancies in patients with PCLs, as well as their family history of malignancies [2–4]. In our study we examine the association between PCLs and other primary malignancies in patients' personal or family history in Greek population. This retrospective study included 144 patients with histopathologically confirmed diagnosis of PCL and 130 controls matched by gender and age (±4 years). All patients and controls completed a detailed questionnaire regarding sun exposure habits-including personal history of sunburns, use of photo-protective measures, use of sunbeds- exposure to ionizing radiation, smoking and alcohol consumption habits, detailed medical personal history (diseases, medication, other chronic skin conditions, personal history of malignancies, etc.) and family history (chronic skin conditions, malignancies). Statistical analysis of the data was performed using PASW Statistics v25 software. Of the 144 patients included, 78 (54.2%) were males and 66 (45.8%) females (median age 57 years old) and among the 130 control subjects, 70 (53.8%) were males and 60 (46.2%) females (median age 61 years old). Analysis showed that family history of solid organ cancer is related to higher relative risk (OR 1.815) of CL development (p<0.05, 95% CI 1.11–2.95). The two types of solid organ tumors more highlighted were liver cancer (p=0.046) and stomach cancer (p=0.002). Family history of skin cancer as well as hematologic malignancies doesn't seem to affect the risk of CL in statistically significant way (p=0.247 and p=0.093 relatively) in our group. As far as the personal history of other primary malignancies is concerned, statistical analysis showed that there might be a correlation between other malignancies and primary cutaneous lymphomas (p<0.05). At this point we should notice the limitation that the number of patients and controls having a personal history of any type of cancer was small, but it consists a predictor. There are several studies analyzing the correlation of PCLs and second primary cancers. The results of our study showed an association of personal history of cancer with PCLs, as well as a correlation of family history of solid organ malignancy with a higher relative risk of cutaneous lymphoma development. No higher relative risk was detected in subjects with family history of hematologic malignancies. We believe that it is important to be aware of the personal and the family history of patients with PCL, as far as the malignancies are concerned, and try to early recognize possible second malignancies in these patients. [ABSTRACT FROM AUTHOR]

Lymphomatoid papulosis (LyP) is a primary lymphoma of the skin with a benign prognosis; it is rare in children and must be differentiated from other primary cutaneous nodular lymphomas, even more rare in children. However, the latter require, unlike LyP, accurate staging and appropriate therapy. On the other hand, the differential diagnosis does not arise with mycosis fungoides, which begins with not infiltrated patches. [ABSTRACT FROM AUTHOR]

We report a case of a woman with diffuse large B-cell lymphoma (DLBCL). Primary cutaneous lymphomas (PCLs) represent distinct clinical and histopathologic subtypes of extranodal T- and B-cell lymphomas. Cutaneous B-cell lymphomas comprise 20-25% of all primary cutaneous lymphomas. The patient presented an erythematous tumour mass of the left nasolabial fold, nose and left cheek as well as disseminated infiltrative plagues on the trunk, arms and left lower leg. Skin biopsy revealed a diffuse infiltrate of lymphocytes around hair follicles and blood vessels within dermis and subcutaneous tissue. An immunohistochemistry showed a diffuse infiltrate of large non-cleaved B-cells, with a high proportion of centroblast-like cells within dermis. Tumor cells expressed CD20, bcl-2 protein and did not express CD10. The patient was misdiagnosed as the erysipelas of the face and unsuccessfully treated with long-term antibiotic therapy by a laryngologist and a dermatologist. The correct diagnosis was delayed and established after 6 months' history of DLBCL lesions. Therefore, we would like to strongly stress the importance of considering diagnosis of cutaneous lymphomas in chronic skin lesions non-responsive to adequate therapies. [ABSTRACT FROM AUTHOR]

Time to next treatment (TTNT) is an emerging endpoint in clinical studies of primary cutaneous T-cell lymphomas (CTCL), with utility as a surrogate marker for the "duration of clinical benefit". TTNT provides a highly clinically meaningful endpoint that uniquely reflects not only the duration of treatment efficacy on disease and symptom control, but also incorporates the patient experience by accounting for patient compliance and tolerance to the studied therapy(s). Given the distinct challenges of pin-pointing the exact date of progression in patients with multi-compartmental CTCL, TTNT overcomes many of the shortcomings of conventional, disease-focused, clinical endpoints in primary CTCL research. Although widely accepted in clinical research for numerous other incurable malignancies, TTNT currently lacks a standardised definition. In this paper, we describe the value of TTNT as a clinical endpoint, review the applications of TTNT in primary CTCL research, and propose a standardised definition of TTNT to be applied in future clinical research of primary CTCL therapies. [ABSTRACT FROM AUTHOR]

Therapeutic progress in primary cutaneous lymphomas continues to be largely dominated by the T-cell lymphomas, towards which the great majority of recent therapeutic innovations have been directed. The latter include local treatments consisting either of relatively classical but "revamped" approaches involving different pharmaceutical forms (example: chlormethine gel) or else lower but seemingly equally effective dosages (electron therapy), or of more innovative approaches (example: UVA-1, dynamic phototherapy, imiquimod, resimiquimod). However, significant progress has been made chiefly in terms of systemic treatments with the emergence of "targeted" drugs that directly and specifically target tumour cells (monoclonal antibodies directed against CD30, CCR4 or CD158k) and the further development of "small" molecules such as histone deacetylase inhibitors and new cytostatics. Immunotherapies, which have proven so effective in other areas of oncodermatology, are also of great interest, while allogeneic haematopoietic stem-cell transplantation has clearly shown its superiority over autologous transplantation and now constitutes a significant component of the therapeutic arsenal in advanced disease. While the innovations in terms of B-cell lymphomas are certainly less significant, mention must also be made of the value of rituximab combined with polychemotherapy (CHOP) and of lenalidomide (as second-line therapy) in primary cutaneous diffuse large B-cell lymphoma, leg type, along with the development of localized (very) low-dose radiotherapy in unilesional or paucilesional indolent forms., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)