Clinical features associated with the invasive component in lentigo maligna of the head and neck: A retrospective study of 175 cases.

BACKGROUND: Lentigo maligna (LM) can develop into lentigo maligna melanoma (LMM) with risk of metastatic dissemination. LMM may be underestimated on the basis of the initial biopsy. The invasion may affect both the therapeutic options and the prognosis. OBJECTIVES: To identify the clinical features associated with invasive forms of LM and factors associated with its recurrence. METHODS: A retrospective, single-centre study of consecutive LM and LMM histologically confirmed and treated by surgery between 2009 and 2014. RESULTS: In total, 175 patients with LM/LMM were surgically treated in our establishment. In men, lesions were more likely to be in the "peripheral zone" (41.8%), while in women they were seen more often in the "central zone" (P=0.001). In multivariate analysis, only the peripheral zone was found to be associated with a risk of invasion (P=0.008). The rate of recurrence was 9% and lesions were more likely to be primary LMM (P=0.0006) excised with clear margins. CONCLUSION: The treatment of choice in LM with non-clear margins must be re-excision, especially for lesions situated in the peripheral zone. Close follow-up is recommended due to risk of recurrence, even in the case of clear margins.

Surgical treatment of rhinophyma: Retrospective monocentric study and literature review.

BACKGROUND: Treatment of rhinophyma consists primarily of destructive procedures. There is currently no consensus regarding treatment. In this study, we propose an algorithm based on a cohort of 25 patients and a literature review. PATIENTS AND METHODS: This was a retrospective study conducted between January 2016 and December 2018. The cosmetic outcome was evaluated by 2 independent assessors based on pre- and postoperative photographs. Patients were ranked according to the severity (mild, moderate, severe) of their rhinophyma. The different surgical methods used were cold blade excision or rhinoshave, electrosurgery or monopolar diathermy knife (MDK), and carbon dioxide laser (CO2 laser), either alone or in combination with another technique. All patients were contacted after the procedure to evaluate their satisfaction and to investigate for adverse effects. RESULTS: Twenty-five patients were included retrospectively: 7 with mild rhinophyma (5 were treated by MDK, 1 by fractional CO2 laser, and 1 by cold-blade excision and TCA solution), 11 with moderate rhinophyma (2 were treated by MDK, 9 by continuous CO2 laser), and 7 with severe rhinophyma (2 were treated by MDK, 5 by MDK plus CO2 laser). Cosmetic outcomes were deemed good or excellent in 80% of cases, and 84% of patients were fully satisfied with the result. We observed 5 cases of hypertrophic scarring, 2 cases of hypopigmentation, 3 cases of notching of the nasal ala, and 7 cases of prolonged erythema, most of which were caused by the MDK technique. CONCLUSION: A wide range of treatment options are available for rhinophyma. We suggest the use of cold-blade excision and trichloroacetic acid or fractional carbon dioxide laser for mild rhinophyma, continuous and pulsed CO2 laser for moderate rhinophyma, and MDK for severe rhinophyma.

Fibrous dysplasia of the orbital region: Series of 12 cases and review of the literature.

PURPOSE: Fibrous dysplasia (FD) is a benign fibro-osseous developmental disorder of growing bone, sometimes involving the craniofacial skeleton (CFD). We wish to present a patient series with CFD of the orbital region and discuss treatment modalities. METHODS: Twelve patients were referred for orbital CFD in the Nantes University Hospital between 2000 and 2018 and studied according to the clinical parameters, radiological features, and modalities of treatment. RESULTS: The mean age was 25.6 years. Ten patients exhibited facial asymmetry with vertical globe dystopia (75%), proptosis (58%) and facial bump (50%). The disease was monostotic in 83% of patients, involving the frontal bone (25%), the sphenoidal bone (33%), the fronto-sphenoidal complex (25%), and the skull base (17%). Unilateral radiological proptosis was found in 7 patients, with a mean protrusion 3.9mm. The optic canal was involved in 75% of patients, with no functional impairment. Three patients were treated with bisphosphonate therapy to stop progression of the disease; 6 patients were given a bone remodelling procedure with good aesthetic outcomes. CONCLUSION: The orbit is a rare localization for FD causing aesthetic and functional disabilities. Medical and surgical treatment can be proposed as part of a multidisciplinary approach.

Effect of parathyroidectomy on quality of life and non-specific symptoms in normocalcaemic primary hyperparathyroidism.

BACKGROUND: Normocalcaemic primary hyperparathyroidism (NcPHPT) is a new clinical entity being diagnosed increasingly among patients with mild primary hyperparathyroidism (PHPT). The aim of this study was to evaluate quality of life and non-specific symptoms before and after parathyroidectomy in patients with NcPHPT compared with those with hypercalcaemic mild PHPT (Hc-m-PHPT). METHODS: This was a prospective multicentre study of patients with mild PHPT from four university hospitals. Patients were evaluated before operation, and 3, 6 and 12 months after surgery for quality of life using the SF-36-v2® questionnaire, as well as for 25 non-specific symptoms. RESULTS: Before operation, the only statistically significant difference between the NcPHPT and Hc-m-PHPT groups was in the mean(s.d.) blood calcium level (2·54 versus 2·73 mmol; P < 0·001). At 1 year after surgery, the blood calcium level had improved significantly in both groups, with no significant difference between them. Quality of life improved significantly in each group compared with its preoperative score, with regard to the physical component summary (P = 0·040 and P = 0·016 respectively), whereas the mental component summary improved significantly in the Hc-m-PHPT group only (P = 0·043). Only two non-specific symptoms improved significantly in the NcPHPT group compared with nine in the Hc-m-PHPT group. CONCLUSION: Parathyroidectomy mildly improves quality of life and some non-specific symptoms in patients with NcPHPT.

Enrichment in c-Kit improved differentiation potential of amniotic membrane progenitor/stem cells.

INTRODUCTION: Human term placenta has attracted increasing attention as an alternative source of stem cells for regenerative medicine since it is accessible without ethical objections. The amniotic membrane (AM) contains at least two stem cell types from different embryological origins: ectodermal amniotic epithelial stem cells, and mesodermal mesenchymal stromal cells. Among the second group we studied the characteristics of amniotic mesenchymal cells (AMC) versus the ones enriched for the commonly used surface marker c-Kit (amniotic progenitor/stem cells-ASC), a stem cell factor receptor with crucial functions in a variety of biological systems and presents in early progenitors of different origin, as been already demonstrated in the enriched chorionic stem cells. METHODS: After isolation, cells from the amniotic membranes (amniotic cells-AC) were selected for c-Kit (ASC) and compared these cells with c-Kit unselected (AMC), evaluating the expression of other stem cell markers (Oct-4, Tra-1-81, SSEA-4), CD271 and Slug. RESULTS: Immunofluorescence analysis showed that ASC cells exhibited greater stem cell marker expression and included more CD271 and Slug positive cells. This was consistent with the interpretation that c-Kit enriched AC show greater stemness capacity compared to c-Kit unselected AMC. DISCUSSION: AMC and ASC can both differentiate into various cell types including adipogenic, osteogenic, chondrogenic, neurogenic and hepatic lineages, but the enrichment in c-Kit improved stemness and differentiation potential of ASC.

Enrichment in c-Kit+ enhances mesodermal and neural differentiation of human chorionic placental cells.

OBJECTIVE: Human term placenta (HTP) has attracted increasing attention as an alternative source of stem cells for regenerative medicine since the amniochorionic membrane harbors stem cells populations that are easily accessible, abundantly available without ethical objections. In the chorionic side of HTP we found a progenitor perivascular "niche" in which rare cells co-express Oct-4 and c-Kit. We investigated the stem cell characteristics and differentiation potential of a chorionic derived population enriched in c-Kit(+) cells and compared this to the unenriched population. STUDY DESIGN: Cells, isolated from the chorion of HTP, were expanded and enriched in c-Kit(+) cells (Chorionic Stem Cells-CSC). Histological staining, immunofluorescence, Western blot and flow cytometry were used to verify the stem cells characteristics of the populations and to compare the differentiation capability towards mesodermal and neural lineages in vitro. RESULTS: The expression of the pluripotent marker Oct-4 was greater in the CSCs compared to the unselected cells (Chorionic Cell-CC) but both Oct-4 and c-Kit expression decreased during passages. After differentiation, CSC displayed stronger chondrogenic and osteogenic potential and a greater adipogenic forming capacity compared to unselected ones. CSC differentiated better into immature oligodendrocytes while CC showed a neuronal progenitor differentiation potential. Moreover, both populations were able to differentiate in hepatogenic lineage. CONCLUSION: CSC display improved Oct-4 expression and a high differentiation potential into mesodermal lineages and oligodendrocytes.

[No interrupted surgical defects of the white upper lip: repair by a combined advancement and rotation flap in the lip subunit]. / Pertes de substance non transfixiantes de la lèvre supérieure blanche: réparation en unité esthétique par lambeau de rotation-avancement intralabial.

A solid understanding of anatomy, basic surgical principles, and tissue movement is essential when undertaking the reconstruction of facial cutaneous surgical defects. Aesthetic facial reconstruction requires understanding ability to use the tissue adjacent to the defect to create a reconstruction that preserves the function of the area and the cosmetic facial units and subunits. The closure of non interrupted white upper lip defects by using a combined advancement and rotation flap is preferred for defects not overtaking 2.5 cm in diameter.

High frequency of vitamin D deficiency in HIV-infected patients: effects of HIV-related factors and antiretroviral drugs.

OBJECTIVES: The aim of this study was to assess 25-hydroxyvitamin D (vitamin D) status in an HIV-infected adult population and to define HIV- and antiretroviral-related factors associated with vitamin D deficiency. METHODS: Using data from a prospective cohort of HIV-infected adult patients followed in five French centres (Dat'AIDS cohort), we evaluated the prevalence of vitamin D deficiency/insufficiency (<30 ng/mL). A multiple linear regression model was used to examine risk factors for vitamin D deficiency (≤10 ng/mL). RESULTS: Vitamin D deficiency/insufficiency was observed in 86.7% of the 2994 patients, including 55.6% with vitamin D insufficiency and 31.1% with vitamin D deficiency. In multivariate analysis, factors associated with vitamin D deficiency were current smoking [adjusted OR (aOR) 1.55], estimated glomerular filtration rate ≥90 mL/min/1.73 m(2) (aOR 1.51), vitamin D measurement not performed in summer (aOR 0.27), CD4 <350 cells/mm(3) (aOR 1.37 for CD4 200 to <350 and 1.62 for CD4 <200 cells/mm(3)) and antiretroviral therapy (aOR 2.61). Gender, body mass index, age, coinfection and previous AIDS were not associated factors. In the antiretroviral-treated population (n = 2660), besides the same factors found in the whole population, efavirenz was the only drug to be significantly associated with deficiency, with an aOR of 1.89 (95% CI 1.45-2.47). CONCLUSIONS: Vitamin D deficiency is frequent in this HIV-infected population. Patients on antiretroviral therapy are at higher risk of vitamin D deficiency than antiretroviral-naive patients, with an increased risk in patients receiving efavirenz. No effect of the other antiretrovirals, including the latest (etravirine, darunavir, raltegravir), was found.

[Refractory thrombotic thrombocytopenic purpura revealing an epiglotis neoplasia]. / Purpura thrombotique thrombocytopénique réfractaire révélant un cancer de l'épiglotte.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with neurological disorder which incidence is relatively rare. Since the discovery of the ADAMTS 13 in 1996 and the changes in the treatment (plasma exchanges and rituximab), the prognosis of the TTP has considerably improved. We report the case of a 60-year-old man with a refractory TTP. The subsequent discovery of an epiglottis neoplasia and its treatment saved the patient.

[Superficial acral fibromyxoma: three cases]. / Fibromyxome acral superficiel : trois observations.

BACKGROUND: in this report of three new cases of superficial acral fibromyxoma (SAF), we discuss the clinicopathological features of this acral fibrous tumour as well as the differential diagnoses for hard nodules affecting the extremities. PATIENTS AND METHODS: these three lesions involved the standard presentation of a large solitary flesh coloured nodule causing deformation of a toenail or a fingernail. Biopsy showed the presence within a fibromyxoid tumour of proliferating fusiform cells having no atypical features and expressing CD 34 diffusely, and in more focal and less consistent fashion, epithelial membrane antigen (EMA) and CD 99. FISH testing for translocation t(17; 22) proved negative. DISCUSSION: this fairly common acral fibrous tumour is poorly known and in certain cases histology results may suggest myxoid dermatofibrosarcoma, which carries a completely different prognosis.

A comparison between bisphosphonates and other treatments for osteoporosis.

Since their development 30 years ago, bisphosphonates are now one of the standard therapy in the management of osteoporosis. Improvements in terms of anti-resorptive potency have leaded to new molecules available either orally or intravenously, from weekly to yearly administration. Overall tolerance of bisphosphonates is good with regards to the risk of mandibular necrosis, not comparable with those observed in cancer treatment, and with no causal link yet established in osteoporotic patients. Compliance remains poor and should be improved by a better education of the patients about their treatment. Other treatments like teriparatide, raloxifene or strontium ranelate are now also available and give more therapeutic options but also more questions on the best molecule to choose for each patient. There is currently no valid basis for distinguishing in a formal and objective manner the different new-generation bisphosphonates, in terms of efficacy against either vertebral, peripheral or hip fractures. In a same way, comparison between bisphosphonates and the other treatments available for osteoporosis is hard in absence of proper randomised controlled study. This review gives an overview of the recent data on the efficacity and tolerance of bisphosphonates in the different forms of osteoporosis and compares them to the other treatments currently available.

[Urachal sinus presenting as umbilical drainage in an adult male]. / Sinus de l'ouraque révélé par une suppuration ombilicale à l'âge adulte.

BACKGROUND: Although urachal remnants are very rare, dermatologists must be aware of them in order to ensure suitable treatment by urologists. Lack of appropriate treatment exposes patients to the risks of recurrent infectious complications or degeneration to vesical adenocarcinoma. CASE REPORT: A 32-year-old man consulted for mild and recurring umbilical weeping for approximately 1 year. He had previously undergone unsuccessful treatment, comprising application of liquid nitrogen and of silver nitrate to an umbilical lesion measuring several millimetres regarded as pyogenic granuloma. The diagnosis of urachal sinus was suspected clinically (umbilical weeping or inflammation) and confirmed by urological scan. DISCUSSION: A defect involving partial or total obliteration of the urachal canal after the fifth month of gestation can result in four types of benign abnormalities. Patent urachus (50%) is diagnosed during the neonatal period, while in adults, the most frequent form is a urachal cyst (30%), with sinuses (15%) and the diverticula (5%) being very rare. The urachus and its implantation base on the bladder must be removed surgically. Laparoscopic surgery offers a valuable alternative to open-surgery and probably leads to less morbidity and shorter convalescence in active young subjects.

[Benign urachus abnormalities: embryology, diagnosis and treatments]. / Pathologies bénignes de l'ouraque chez l'adulte : origine embryologique, présentation clinique et traitements.

Urachus pathologies are very rare but require to be known by urologists. Lack of appropriate treatment exposes the patients to the risks of symptoms recurrence, infectious complications or adenocarcinomatous degeneration. A partial or total defect of obliteration of the urachus channel after the fifth month of gestation can be at the origin of four benign pathologies. The ombilicovesical fistula (47.6%) is diagnosed at the native period. In the adult, the most frequent form is the cyst (30.7%) whereas the external (16.4%) and internal sinus (3.2%) are rarer. Diagnosis depends on the clinical examination and the association of sonography and TDM. The risk of complications must systematically result in proposing a surgical treatment for these benign pathologies. The umbilicus resection is not recommended, but the surgeon has to remove the urachus and its implantation base on the bladder. Laparoscopic surgery seems to be an interesting route for this intervention.

Transplantation of human fetal mesenchymal stem cells improves glomerulopathy in a collagen type I alpha 2-deficient mouse.

Fetal mesenchymal stem cell (fetal MSC) therapy has potential to treat genetic diseases with early onset, including those affecting the kidney and urinary tract. A collagen type I alpha 2-deficient mouse has a deletion in the alpha2 chain of the procollagen type I gene, resulting in the synthesis of abnormal alpha1(I)(3) homotrimers, which replace normal alpha 1(I)2 alpha 2(I)1 heterotrimers and a glomerulopathy. We first confirmed that col1 alpha 2-deficient homozygous mice show abnormal collagen deposition in the glomeruli, which increases in frequency and severity with postnatal age. Intrauterine transplantation of human MSCs from first trimester fetal blood led postnatally to a reduction of abnormal homotrimeric collagen type I deposition in the glomeruli of 4-12 week-old col1 alpha 2-deficient mice. Using bioluminescence imaging, in situ hybridization and immunohistochemistry in transplanted col1 alpha 2-deficient mice, we showed that the damaged kidneys preferentially recruited donor cells in glomeruli, around mesangial cells. Real-time RT-PCR demonstrated that this effect was seen at an engraftment level of 1% of total cells in the kidney, albeit higher in glomeruli. We conclude that intrauterine transplantation of human fetal MSCs improves renal glomerulopathy in a collagen type I-deficient mouse model. These data support the feasibility of prenatal treatment for hereditary renal diseases.

Infection control practices and infectious complications in dermatological surgery.

The aim of this study was to assess infection control practices and their impact upon infectious complications in skin surgery conducted by private dermatologists. A prospective study was carried out by 73 volunteers belonging to the Surgical Group of the Société Française de Dermatologie over a period of three months. Data were collected for surgical procedures performed during this period, including the excision of all benign or malignant tumours, but excluding sebaceous cysts and pyodermas. A total of 3491 dermatological surgical procedures were included in the survey. Post-operative infections occurred in 67 patients (1.9%), with superficial suppuration accounting for 92.5% of surgical site infections. The incidence was higher in the excision group with a reconstructive procedure (4.3%) than in excisions alone (1.6%). Infection control precautions varied according to the site of procedure; multivariate analysis showed that haemorrhagic complications were an independent factor for infection in both types of surgical procedure. The male gender, immunosuppressive therapy and not wearing sterile gloves were independent factors for infections occurring following excisions with reconstruction. Not all of the procedures needed the use of a hospital theatre. It is clear that for excisions with a reconstructive procedure or for certain anatomical sites, such as the nose, there should be more emphasis upon infection control precautions. Further studies are needed to establish optimal guidelines for this kind of surgery.

A prospective study of the incidence of complications associated with dermatological surgery.

BACKGROUND: Dermatological surgery is a relatively new and expanding subspecialty within dermatology. Little information is available about complications in this kind of surgery in the European setting. OBJECTIVES: The aim of this study was to assess the incidence of anaesthetic, haemorrhagic and infectious complications in dermatological surgery and to highlight the factors associated with these complications. METHODS: Data were collected prospectively over a 3-month period for all surgical procedures performed by a network of dermatologists (n = 84 dermatologists) in France, including the excision of all benign or malignant tumours but excluding sebaceous cysts and pyodermas. Information was collected regarding dermatologists, patients, procedures and complications. RESULTS: A total of 3788 surgical procedures were available for review; 236 complications, mostly minor, occurred in a total of 213 surgical procedures (6%), bleeding being the most common (3%). Vaso-vagal syncope was the main anaesthetic complication (51 of 54). Infectious complications occurred in 79 patients (2%). Superficial suppuration accounted for 92% of surgical site infections. Only one patient had a systemic infection. Complications requiring additional antibiotic treatment or repeat surgery accounted for only 22 cases of 3788 (1%). No statistically significant correlation was found with the characteristics of the dermatologists, especially with respect to their training or amount of surgical experience. Similarly, no link could be established between complications and surgical conditions. Multivariate analysis showed that anaesthetic or haemorrhagic complications were independent factors for infectious complications. Sex, administration of an anticoagulant or immunosuppressant, type of procedure performed and duration exceeding 24 min were independent factors for haemorrhagic complications. CONCLUSIONS: This study shows a low rate of complications associated with dermatological surgery performed by dermatologists under local anaesthesia on an outpatient basis.