CXCL13 is a key B cell chemoattractant and marker of disease activity in patients with SLE; however, the mechanism of its induction has not been identified yet. Here, we have shown that the proteoglycan biglycan triggers CXCL13 expression via TLR2/4 in macrophages and dendritic cells. In vivo, levels of biglycan were markedly elevated in the plasma and kidneys of human SLE patients and lupus-prone (MRL/lpr) mice. Overexpression of soluble biglycan in MRL/lpr mice raised plasma and renal levels of CXCL13 and caused accumulation of B cells with an enhanced B1/B cell ratio in the kidney, worsening of organ damage, and albuminuria. Importantly, biglycan also triggered CXCL13 expression and B cell infiltration in the healthy kidney. Conversely, biglycan deficiency improved systemic and renal outcome in lupus-prone mice, with lower levels of autoantibodies, less enlargement of the spleen and lymph nodes, and reduction in renal damage and albuminuria. This correlated with a marked decline in circulating and renal CXCL13 and a reduction in the number of B cells in the kidney. Collectively, our results describe what we believe to be a novel mechanism for the regulation of CXCL13 by biglycan, a host-derived ligand for TLR2/4. Blocking biglycan-TLR2/4 interactions might be a promising strategy for the management of SLE and other B cell-mediated inflammatory disease entities.

Defects of the perineum may result from ablative procedures of different malignancies. The evolution of more radical excisional surgery techniques resulted in an increase in large defects of the perineum. The perineogenital region per se has many different functions for urination, bowel evacuation, sexuality, and reproduction. Up-to-date individual and interdisciplinary surgical treatment concepts are necessary to provide optimum oncological as well as quality of life outcome. Not only the reconstructive method but also the timing of the reconstruction is crucial. In cases of postresectional exposition of e.g., pelvic or femoral vessels or intrapelvic and intra-abdominal organs, simultaneous flap procedure is mandatory. In particular, the reconstructive armamentarium of the plastic surgeon should include not only pedicled flaps but also free microsurgical flaps so that no compromise in terms of the extent of the oncological resection has to be accepted. For intra-abdominally and/or pelvic tumors of the rectum, the anus, or the female reproductive system, which were resected through an abdominally and a sacrally surgical access, simultaneous vertical rectus abdominis myocutaneous (VRAM) flap reconstruction is recommendable. In terms of soft tissue sarcoma of the pelvic/caudal abdomen/proximal thigh region, two-stage reconstructions are possible. This review focuses on the treatment of perineum, genitals, and pelvic floor defects after resection of malignant tumors, giving a distinct overview of the different types of defects faced in this region and describing a number of reconstructive techniques, especially VRAM flap and pedicled flaps like antero-lateral thigh flap or free flaps. Finally, this review outlines some considerations concerning timing of the different operative steps.

No abstract

We present the case of a 44-year-old female with an aggressive digital papillary adenocarcinoma of the right thumb. Aggressive digital papillary adenocarcinomas are rare tumours originating from sweat glands of the skin on the hand or foot. The tumour was located at the extensor side and had been excised in 2 operations after diagnosis as an eccine hidradenoma, which is exceptional in this case. The tumour was radically excised and the defect was reconstructed using arthrodesis, and a retrogradely perfused pedicle radialis fascial flap with skin graft, due to which amputation was avoided.

Summary:Complex fracture dislocations of the wrist often result in posttraumatic arthrosis. In trying to avoid total arthrodesis, alternative treatment strategies have been investigated. For this purpose, we present the case of a 56-year-old male patient who sustained a direct trauma during a circular saw accident, resulting in the destruction of the distal carpal row among other things. However, the proximal carpal row was found completely intact. We indicated an emergency distal row carpectomy. Eighteen months postoperatively, the patient showed very good range of movement with no pain, and radiologically, the proximal carpal row was still intact, with no signs of an incipient radiocarpal arthrosis. This case demonstrates the successful removal of the distal carpal row in terms of a distal row carpectomy.