Search

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.

Summary Background Dermatitis artefacta (DA) is a dermatologicopsychiatric illness that is a conscious self-infliction of lesions to accessible regions of the body. The lesions usually do not resemble those of any know skin disease and there are no specific diagnostic tests to recognize them. This makes dermatitis artefacta a very slow, challenging and expensive disease to diagnose. Case Report We present 5 different clinical cases of dermatitis artefacta treated in the Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk in 2011. Detailed anamnesis and physical examination were performed at the day of admission. All patients had biochemical and hematological blood tests, skin biopsies and swabs for bacteriological examination, and photographs were taken. Psychiatric consultation was recommended in all cases. Clinical symptoms before diagnosis lasted from 1 to 10 years. The female-to-male ratio is 1:0.7, with age range of 57–62 years. Of our patients, only 2 refused a psychiatric consultation. Three out of 5 patients denied self-mutilation (2 of those 3 patients finally admitted to self-manipulations). Lesions were usually within the reach of the dominant hand. Two patients have other personality disorders. In 4/5 cases visible improvement after treatment with occlusive dressings were observed. Conclusions We discuss and attempt to depict issues associated with collaboration between dermatologists and psychiatrists, reasons for poor recognition of the disease, very long diagnosis and high costs. To conclude, we found that close collaboration between dermatologists and psychiatrists is important in diagnosing and treating DA patients.