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Background: Mycosis fungoides (MF) and Sezary syndrome (SS) are forms of primary cutaneous T-cell lymphomas. The clinical aspects and symptoms vary greatly depending on the stage of disease. These are rare diseases which remain incurable, but there is a wide variety of treatment options. Few studies have been performed on this topic in Sweden and there is an emerging need for descriptive studies and research in this field. Aim: The aim of this study is to describe and analyze diagnostic criteria for patients with MF/SS and to describe treatment modalities and evaluate treatment response of this patient-group. Method: This is a descriptive retrospective study based on the data collected in the Primary Cutaneous Lymphoma quality register at the department of Dermatology and Venerology at Sahlgrenska University Hospital between 1997-2021. Results: Eighty-two patients, median age 55.9 years (range 8.6–91.7) at diagnosis, with MF (n=70) or SS (n=12) were included. Male to female ratio was 1.7:1. From the onset of skin symptoms until initial diagnosis took on average 2.7 years (median, range 0.1–42.4 years). The subtypes of MF were folliculotropic (n=9), syringotropic (n=4), hypopigmented (n=1) and hyperpigmented (n=1). The majority of the patients (n=63) were diagnosed at early-stage disease (IA-IIA) while 18 patients were diagnosed with advanced stage disease (IIB–IVB). Overall disease progression was seen in 10% (n=8) of the patients. Eight (10%) patients were deceased out of which three (3.8%) deaths were associated with MF/ SS related causes. The most common co-morbidities in our study population were overweight 31%, hypertension (29%), obesity (18%), hyperlipidaemia (16%), diabetes mellitus (15%), and dermatitis (11%). The most common malignancies were basal cell carcinoma (16%), haematological malignancies (7%), malignant melanoma (6%) and prostate cancer (6%). Topical corticosteroids (96.3%), UVB (57.3%) and PUVA (47.6%) were common choices of treatment in all patients, along with acitretin in those with advanced stage disease. No significant difference in overall response was detected between patients with early-stage disease and advanced stage disease for any treatment. Conclusion: This study contributes to knowledge on epidemiological and clinical features in addition to diagnostic findings in patients with MF and SS diagnosed and/or followed up for a long period of time in Western Sweden. We observed increased risk of cardiovascular disease and higher risk of developing other malignancies. This study suggests that treatment for these patients have been adequately adjusted for stage and highlights the importance of topical corticosteroids as an integrated part of the treatment. UVB and PUVA are beneficial treatments for patients with early-stage disease, but could also be valuable for patients with advanced disease along with acitretin. Hopefully this study will inspire to and be integrated in future research ahead.

Background: Primary cutaneous B-cell lymphomas (PCBCLs) are a group of commonly indolent diseases that comprises primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT). PCBCLs affect mainly adults but also children. Treatment is watchful waiting, excision, radiotherapy, rituximab, and systemic chemotherapy in case of extracutaneous spread disease. The disease is rare and there is a huge need of studies on clinical features and treatment outcomes. Aim: The aim of the study was to present demographics, clinical features and treatment outcomes of patients with PCBCLs in West Sweden. Methods: This is a descriptive, register-based, single center retrospective study of patients diagnosed with PCBCLs at Department of Dermatology and Venerology at Sahlgrenska University Hospital between 2002-2021. Data regarding demographics, clinical characteristics, pathological- and medical examination findings, comorbidities, staging according to TNM-system and treatment response were collected from patients’ medical records and the hospital-based skin lymphoma register. Results: Nine patients with PCBCLs were identified and included in the study. Mean age at diagnosis was 53.8±14.5 years for patients with PCFCL (n=5) and 49.2±28.1 for PCMZL (n=4), respectively. Skin presentations of PCFCL were in form of papules, plaques and tumors (40%) preferably distributed on the head (60%) and trunk (40%). Papules (75%) on upper extremity (50%) were the most common manifestation in PCMZL. In most cases the histopathological diagnosis was set after the first pathological anatomical diagnosis but for PCFCL it required two or three biopsies before the diagnosis was confirmed. Monoclonality was identified in 20–25% in PCFCL and PCMZL. Lactate dehydrogenase was increased at any time in PCFCL 40% and PCMZL 25%. All the patients with PCFCL were tested for borreliosis and all were negative. One of two PCMZL patients was tested positive for borreliosis. Enlarged lymph node was found in 20% of the patients with PCFCL, all had done a CT. Radiotherapy, excision, topical/intralesional corticosteroids, chemotherapy and rituximab were used to treat PCBCLs. PCMZL had complete remission when treated with chemotherapy, radiotherapy and with excision. The treatments were commonly used as monotherapy or in a combination over time. The study population had a wide range of different comorbidities including cardiovascular diseases, hypothyroidism, rheumatic arthritis and dermatological diseases including malignant melanoma, basal cell carcinoma and herpes zoster. Conclusion: This study contributes to increased knowledge on demographics, clinical features, diagnostics, and treatment response of PCBCLs. The results of the study can be used in future research and adds some new knowledge about these unusual diseases in clinical practice.