In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sézary syndrome (SS), the European Organisation for Research and Treatment of Cancer-Cutaneous Lymphoma Task Force (EORTC-CLTF) published in 2006 its consensus recommendations for the stage-adapted selection of management options for these neoplasms. Since then, the understanding of the pathophysiology and epidemiology of MF/SS has advanced, the staging system has been revised, new outcome data have been published and novel treatment options have been introduced. The purpose of the present document is to update the original recommendations bearing in mind that there are still only a limited number of controlled studies to support treatment decisions for MF/SS and that often treatment is determined by institutional experience and availability.
View Article and Find Full Text PDFWe describe a patient with a 30-year history of mycosis fungoides (MF) and renal transplantation performed 3 years before he presented with an ulcerated tumour in the lumbosacral area. Biopsy revealed a lymphatic infiltrate of medium-sized, pleomorphic T cells expressing the gamma-delta T-cell receptor. Radiological staging and bone marrow biopsy revealed no extracutaneous involvement.
View Article and Find Full Text PDFBackground: Mast cells (MC) are bone marrow derived haematopoetic cells playing a crucial role not only in immune response but also in the tumor microenvironment with protumorigenic and antitumorigenic functions. The role of MC in primary cutaneous T-cell lymphomas (CTCL), a heterogeneous group of non-Hodgkin lymphomas with initial presentation in the skin, is largely unknown.
Objective: To gain more accurate information about presence, number, distribution and state of activation (degranulated vs.
There is currently no information available on illness perception in primary cutaneous T-cell lymphomas (CTCL). The aim of this study was therefore to gather initial information on disease understanding and interpretation in patients with CTCL. Consecutive patients from a hospital-based primary cutaneous lymphoma ward completed the Revised Illness Perception Questionnaire (IPQ-R) on 2 consecutive visits.
View Article and Find Full Text PDFBackground: Non-melanoma skin cancer (NMSC), including squamous cell carcinoma (SCC) and basal cell carcinoma (BCC), is the most common malignancy. Unresectable or metatstatic SCC is rare and therapy can be difficult because of advanced age and comorbidities. Targeted therapy with monoclonal antibodies (mAbs) against EGFR is an option in these patients.
View Article and Find Full Text PDFThe vascular type of the Ehlers-Danlos syndrome (Ehlers-Danlos syndrome type IV, EDS IV; OMIM #130050) is a rare connective tissue disorder with autosomal dominant transmission caused by mutations in the COL3A1 gene resulting in increased fragility of connective tissue with arterial, intestinal, and uterine ruptures and premature death. We present a 28-year-old female who in addition to typical EDS IV symptoms had severe peripheral artery occlusive disease (PAOD) and subtotal stenosis of the abdominal aorta. COL3A1 sequencing resulted in detection of an as yet undescribed mutation in exon 36 at position 2465 leading to a nucleotide replacement (c.
View Article and Find Full Text PDFBackground: Procalcitonin (PCT) is a specific biomarker for early detection of bacterial infections. While the usefulness of procalcitonin in severe conditions such as sepsis is well established, its relevance in the diagnosis and prognosis of localized cutaneous bacterial infections is unknown. Our aim was to initially evaluate if PCT is a useful parameter for predicting the severity of skin and skin structure infections (SSSI).
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