[Mycosis fungoides in a heart transplant recipient]. / Mycosis fongoïde après transplantation cardiaque.

BACKGROUND: Skin cancer occurs frequently in organ transplant patients as a result of induced immunosuppression. Most cases involve carcinomas or B-cell lymphomas induced by the Epstein Barr virus (EBV). Cutaneous T-cell lymphomas remain rare. We report a case of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant recipient. PATIENTS AND METHODS: A 68-year-old man who had received a heart transplant 21years earlier and was being treated with tacrolimus, mycophenolate mofetil and prednisolone had been presenting a psoriasiform rash on his trunk, limbs and head for 4years. The rash was resistant to both PUVA therapy and topical corticosteroids. Histopathological examination suggested epidermotropic cutaneous T-cell lymphoma. There was no impairment of the patient's general state of health nor any adenopathy. Molecular biology revealed TCR rearrangement in both blood and skin. Screening for circulating Sézary cells was negative, and PET scan revealed no signs of extracutaneous localization. Mechlorethamine showed little efficacy, bexarotene was complicated by dysthyroidism, hypertriglyceridemia was ineffective, methotrexate was contraindicated because of calcineurin inhibitor-related chronic kidney failure, and interferon could not be given due to the context of heart transplantation. Finally, we treated our patient with gemcitabine, which initially proved effective but was later complicated by septic shock that resulted in the patient's death. CONCLUSION: The particularities of our observation are the onset of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant patient, and the therapeutic difficulties encountered in a setting of transplantation with immunodepression.

Factors associated with the choice of the first biologic in psoriasis: real-life analysis from the Psobioteq cohort.

BACKGROUND: Decision-making is a complex process. The aim of our study was to assess factors associated with the choice of the first biological treatment in patients with moderate-to-severe psoriasis. METHODS: Data on all patients included in the French prospective, observational, cohort, Psobioteq and initiating a first biologic prescription between July 2012 and July 2016 were analysed. Demographic information and clinical features were collected during routine clinical assessments by the dermatology team at the recruiting centres using a standardized case report form. The primary outcome was the nature of the first biologic treatment. Four groups were identified as follows: adalimumab, etanercept, ustekinumab and infliximab groups. Factors associated with the choice of the first biological agent were determined by a multinomial logistic regression model adjusted on year of inclusion. RESULTS: The study population included the 830 biological-naïve patients who initiated a first biological agent. The mean age was 46.6 years (±SD 13.9), and 318 patients (38.3%) were female. The most commonly prescribed biologic was adalimumab: 355 (42.8%) patients, then etanercept (n = 247, 29.8%), ustekinumab (n = 194, 23.4%) and infliximab (n = 34, 4.0%). In the multinomial logistic regression analysis, patients were significantly more likely to receive adalimumab if they had a severe psoriasis as defined by baseline PASI or if they had psoriatic arthritis compared to etanercept (aOR, 0.42; 95% CI, 0.16-1.07) and ustekinumab (aOR, 0.15; 95% CI, 0.04-0.52). Patients were significantly more likely to receive ustekinumab (aOR, 2.39; 95% CI, 1.04-5.50) if they had a positive screening for latent tuberculosis compared to adalimumab. Younger patients were also more likely to receive ustekinumab. Patients with chronic obstructive pulmonary disease were more likely to be prescribed ustekinumab or etanercept compared to adalimumab. There was a trend in favour of etanercept prescription in patients with cardiovascular comorbidities, metabolic syndrome and in patients with a history of cancer. CONCLUSION: We identified patient- and disease-related factors that have important influence on the choice of the first biological agent in clinical practice. Clinicians appear to have a holistic approach to patient characteristics when choosing a biological agent in psoriasis.

Systemic sclerosis and exposure to heavy metals: A case control study of 100 patients and 300 controls.

OBJECTIVE: This case control study assessed: 1) the relationship of systemic sclerosis (SSc) related to exposure to heavy metals; and 2) the risk of SSc related to occupational exposure in male and female patients. METHODS: From 2005 to 2008, 100 patients with a definite diagnosis of SSc were included in the study; 3 age, gender, and smoking habit matched controls were selected for each patient. All SSc patients and controls underwent detection and quantification of heavy metal traces in hair samples, using multi-element inductively coupled plasma mass spectrometry (ICP-MS). RESULTS: SSc patients exhibited higher median levels of the following metals: antimony (p=0.001), cadmium (p=0.0003), lead (p=0.02), mercury (p=0.02), molybdenum (p=0.04), palladium (p<0.0001) and zinc (p=0.0003). A marked association between SSc and occupational exposure was further found for: 1) antimony (p=0.008) and platinum (p=0.04) in male patients; and 2) antimony (p=0.02), cadmium (p=0.001), lead (p=0.03), mercury (p=0.03), palladium (p=0.0003) and zinc (p=0.0001) in female patients CONCLUSION: The results show the impact of occupational risk factors in the development of SSc for: antimony, cadmium, lead, mercury, molybdenum, palladium and zinc. Thus, occupational exposure should be systematically checked in all SSc patients at diagnosis. Finally, the association between SSc and occupational exposure may be variable according to patients' gender.

Metabolic comorbidities and hypertension in psoriasis patients in France. Comparisons with French national databases.

INTRODUCTION: Several studies have shown a high prevalence of cardiovascular and metabolic comorbidities in psoriasis. Our study aimed to evaluate the association of psoriasis with key comorbidities such as smoking, obesity, hypertension, dyslipidaemia and diabetes comparatively with French national data. MATERIAL AND METHODS: This multicentre noninterventional observational study of adults with psoriasis was conducted in 29 dermatology centres in France. A total of 2210 patients were included. The prevalence of comorbidities in psoriatic patients was compared to data from the French national databanks "ObEpi 2012" (obesity, hypertension, dyslipidaemia and diabetes) and "Baromètre Santé 2010" (smoking). RESULTS: We reported a higher prevalence of all metabolic comorbidities and high blood pressure in psoriatic patients. Smoking: 32.5% were active smokers; the age of onset and the prevalence of familial psoriasis were significantly lower in the smoking group but the severity of psoriasis was significantly higher. The frequency of smoking was higher than in the general population, particularly among young female patients. Obesity: 24% of patients with psoriasis were obese. Multivariate analysis showed obesity to be significantly associated with other comorbidities, severity of psoriasis and psoriatic arthritis. The incidence of obesity was higher than in general population, occurring chiefly in subjects aged over 45 years. HYPERTENSION: 26% of patients with psoriasis had hypertension. The age of onset of psoriasis and the prevalence of psoriatic arthritis were significantly higher in the hypertension group, although there was less familial psoriasis. The incidence of hypertension was higher than in general population. Dyslipidaemia: 27.5% of patients with psoriasis had dyslipidaemia. The age of onset in the dyslipidaemia group was higher although there was less familial psoriasis. The incidence of dyslipidaemia was higher than in general population. Diabetes: 11.0% of patients with psoriasis had diabetes. The age of onset of psoriasis was significantly higher in the diabetes group although there was less familial psoriasis. The incidence of diabetes was higher than in general population particularly after the age of 35 years. CONCLUSION: These results confirmed that psoriasis is associated with significant metabolic comorbidities and hypertension compared to the general population in France, with certain epidemiological differences for each.

Evaluation of risk factors for body weight increment in psoriatic patients on infliximab: a multicentre, cross-sectional study.

BACKGROUND: A significant weight gain has been reported in patients with psoriasis treated with anti-tumour necrosis factor-alpha agents. Among these patients, there are contradictory results about risk factors for weight gain. OBJECTIVE: Assessing risk factors for weight increment in psoriatic patients on infliximab (IFX). METHODS: This study was a 4-month, non-interventional, cross-sectional, multicentre study on adults with psoriasis performed in 19 French dermatological centres. All the patients who received IFX for at least 1 year were prospectively included, with retrospective analysis of data. Impact of sex, age, severity of the disease, cardiovascular and metabolic comorbidities, and previous and simultaneous systemic treatments on weight changes, was analysed. Weight gain was defined as an increment of more than 2% of baseline weight. RESULTS: Overall, 191 psoriatic patients (males: 68.6%; mean age: 46.9 years) were included. Mean weight gain was 1.6 kg (2.1%) after 1 year of IFX. Half (48.2%) suffered from a weight gain, and 9.9% from a weight increment of 10% or more. Baseline weight and Body Mass Index, and cardiovascular and metabolic comorbidities did not influence weight. Men (P=0.007) and patients with severe psoriasis (BSA, P=0.005) had a tendency to put on weight. Patients with a hospital dietary follow-up (P=0.01; OR=0.36 [0.16-0.79]) and patients on methotrexate (P=0.03; OR=0.41 [0.18-0.93]) during IFX treatment are thinner, in a multivariate analysis. CONCLUSION: Severe weight increment is frequent on IFX treatment, mainly in men, and patients with severe psoriasis. Dietary follow-up or simultaneous use of methotrexate could limit this weight increment.

Prospective study to evaluate the association between systemic sclerosis and occupational exposure and review of the literature.

INTRODUCTION: Systemic sclerosis (SSc) has complex pathogenesis and likely multifactorial causes. Environmental exposures have been suggested to play a role in SSc pathogenesis, including occupational exposure to pollutants and chemicals as well as use of drugs leading to modulation of immune response. Thus, this case-control study aimed to assess: the relationship between SSc and occupational exposure; and the risk of SSc related to occupational exposure in male and female patients. METHODS: From 2005 to 2008, 100 patients with a definite diagnosis of SSc were included in the study; 3 age, gender, and smoking habits matched controls were selected for each patient. A committee of experts evaluated blindly occupational exposure to crystalline silica, white spirit, organic solvents, ketones, welding fumes, epoxy resins, and pesticides; an occupational exposure score was calculated for all subjects. Our findings were compared with previous data in the literature. RESULTS: Increased ORs for SSc were found for: crystalline silica (p<0.0001), white spirit (p<0.0001), aromatic solvents (p=0.0002), chlorinated solvents (p=0.014), trichlorethylene (p=0.044), ketones (p=0.002) and welding fumes (p=0.021). Elevated risk associated with high final cumulative score in SSc was observed for: crystalline silica, white spirit, chlorinated solvents, trichlorethylene, aromatic solvents, any type of solvents, ketones and welding fumes. A marked association between SSc and occupational exposure was further found for: 1) crystalline silica, chlorinated solvents, trichloroethylene, white spirit, ketones and welding fumes in male patients; and 2) white spirit, aromatic solvents, any type of solvent and ketones in female patients. Finally, we did not find an association between SSc and: 1) the use of drugs that have been speculated to play a role in SSc onset (anorexigens, pentazocine, bromocriptine, l-tryptophan); 2) implants - that are prosthesis, silicone implants, and contact lenses; and 3) dyeing hair. In the literature, SSc has been associated with occupational exposure to silica and solvents, while the association between SSc and specific organic solvents and welding fumes has been anecdotally reported. CONCLUSION: The following occupational factors have an impact in the development of SSc: crystalline silica, white spirit, aromatic solvents, chlorinated solvents, trichlorethylene, ketones and welding fumes. The risk of SSc appears to be markedly associated with high cumulative exposure. Finally, the association between SSc and occupational exposure may be variable according to gender.

[Miescher's cheilitis and lymphocytic clonal expansion: 2 cases]. / Macrochéilite de Miescher et expansion lymphocytaire monoclonale. Deux cas.

INTRODUCTION: Melkersson Rosenthal's syndrome is a rare disease that classically combines: orofacial edema, peripheral facial paralysis and a plicated tongue. Miescher's cheilitis represents the monosymptomatic form of the disease. Its etiopathogenesis is unknown. We report 2 cases of Miescher's cheilitis during which the discovery of a monoclonal lymphocyte expansion raised the question of an eventual link between these two diseases. CASE REPORTS: CASE No 1. A 30 Year-old man, without medical past history, had been followed up for 3 Years for Miescher's cheilitis. The supplementary examinations permitted elimination of an infectious cause, Crohn's disease, sarcoidosis or a contact allergy. A serum monoclonal IgG kappa was discovered fortuitously. An X-ray of the skeleton and the myelogram were normal. There was no detectable monoclonal rearrangement of the genes of the blood or bone marrow T or B-cell lymphocyte receptor. In the absence of progression towards a malignant blood disease three Years later, we concluded in a benign monoclonal gammapathy. CASE No 2. A 36 Year-old Algerian man, without past medical history, had been followed-up for 8 Years for a granulomatous macrocheilitis. The search for Crohn's disease, sarcoidosis or a contact allergy was negative and the diagnosis of an incomplete Melkersson Rosenthal syndrome was retained. The blood count revealed persisting hyperlymphocytosis in the blood. The etiological search for a hyperlymphocytosis showed a monoclonal rearrangement of the T-cell lymphocyte receptor genes in the blood lymphocytes. The myelogram was normal. COMMENTS: Melkersson Rosenthal's syndrome is a rare granulomatous disease of the mucosa of the mouth. The etiopathogenesis of this affection is unknown and controversial, several case reports suggest that it could be a disease of immunological origin. A clonal T-cell lymphocyte population was revealed in the labial lesions of a 12 Year-old patient presenting with Melkersson Rosenthal's syndrome during a control visit, without the role of this lymphocyte population having been determined. We report two other cases associating blood lymphocyte proliferation and Melkersson Rosenthal' syndrome. This association is not necessarily fortuitous because of the rarity of the syndrome on the one hand and the uncommon nature of the detection of lymphocyte clones in young patients on the other. The presence of a clonal population can be interpreted in two manners: it can demonstrate chronic antigen stimulation, which with a super-antigenic effect leads to the expansion of a lymphocyte population making it detectable. The other hypothesis would be an increased secretion of cytokines by the lymphocyte clone provoking a granulomatous organization, as during granulomatous lymphomas.

[Evaluation of the diagnosis of pigmented tumors of the skin and factors leading to a decision to excise. Dermatologists of the Postgraduate Association of Haute-Normandie]. / Evaluation du diagnostic des tumeurs pigmentées de la peau et des éléments conduisant à une décision d'exérèse. Dermatologues de l'Association de FMC de Haute-Normandie.

INTRODUCTION: The necessity of excising melanomas characterized by a slight thickness at an early stage, leads dermatologists to remove pigmented lesions which do not correspond to melanomas. The aims of this study were: a) to prospectively assess the accuracy of melanoma diagnosis, b) to quantify the number of excisions performed according to the degree of melanoma suspicion, c) to determine the specific clinical sign or signs of relevant diagnostic value. PATIENTS AND METHODS: This study was conducted prospectively from January 1996 to August 1997 by dermatologists in private practice and dermatologists from a University Hospital staff. When it was decided to excise a pigmented lesion, a form was filled out choosing the most appropriate clinical diagnosis, the degree of melanoma suspicion, and clinical signs which lead to surgery. Based on histological findings as the reference, the sensitivity, specificity, accuracy of melanoma diagnosis and the kappa test that evaluates the concordance between clinical and histological diagnosis, were performed. The diagnostic value of clinical signs was assessed by variance analysis. RESULTS: Of the 353 excised lesions, 38 (10.7 p. 100) were identified as melanoma on histologic examination. The sensitivity, the specificity and diagnostic accuracy were: 79 p. 100, 94 p. 100 and 53 p. 100 respectively. The kappa test concordance between clinical and histological diagnosis was 0.66. Two hundred and two lesions (57 p. 100) were excised even though the clinical suspicion of melanoma was poorly considered. Only one of these 202 lesions was identified histologically as a true melanoma. Thirty seven (24.5 p. 100) of the 151 remaining excised lesions with an "average" or "strong" suspicion were true melanomas. The clinical signs considered, alone or associated, had a poor predictive positive value (< 38 p. 100). An analytical approach performed with a logistic model permitted the identification of two associated signs suggesting a best diagnostic value. DISCUSSION: This is the only study, to our knowledge, reported in the literature which prospectively assesses the sensitivity, specificity and concordance between clinical and histological diagnosis of melanoma. Results were considered from average to good. The originality of this study was to assess the number of pigmented lesions excised according to the degree of melanoma suspicion, suggesting the possibility of reducing the number of nevi removed when the melanoma risk was considered clinically poor. Finally, this study emphasizes the limits of clinical semiology and the need for future diagnostic methods in the assessment of melanoma.