E. Puzenat

What are the best outcome measures for assessing plaque psoriasis severity? A systematic review of the literature.

Background  A wide variety of scoring systems have been proposed to assess severity of psoriasis. Given its importance as a health issue both for patients and health care systems, it is critically important to evaluate the validity and reliability of existing outcome measures.

Cardiovascular risk factors in patients with plaque psoriasis: a systematic review of epidemiological studies.

Introduction  Many epidemiological studies have associated psoriasis with an increased risk of coronary artery disease, resulting from a higher prevalence of cardiovascular risk factors in psoriasis patients compared with unmatched controls. However, the results of epidemiological studies vary depending upon the populations studied. The aim of this systemic review was to evaluate the risk of diabetes, hypertension, dyslipidaemia and obesity in adults with plaque psoriasis. In addition, we assessed the relationship between the risk of cardiovascular risk factors and psoriasis severity.

What are the best outcome measures for assessing quality of life in plaque type psoriasis? A systematic review of the literature.

Background  The assessment of health‐related quality of life (QOL) is important in psoriasis. Despite this, among the wide variety of QOL questionnaires used in psoriasis, there is no consensus as to which is the best.

Assessment of risk of psoriatic arthritis in patients with plaque psoriasis: a systematic review of the literature.

Objective  The aim of this study was to determine the prevalence of psoriatic arthritis (PsA) in the patients with plaque psoriasis and to give recommendation for the diagnosis of PsA for dermatologists.

Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002

Background  Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumour of the skin, with an estimated incidence of 0.8 to five cases per 1 million people per year.

Evidence-based recommendations to assess psoriasis severity: systematic literature review and expert opinion of a panel of dermatologists

Background  Severity of psoriasis appears to be multidimensional and its assessment in everyday clinical practice requires a complex holistic approach.

Systematic search for neutropenia should be part of the first screening in patients with poikiloderma

Poikiloderma occurs in a number of hereditary syndromes, the best known of which is Rothmund-Thomson syndrome (RTS). Differential diagnoses include Dyskeratosis Congenita (DC) with high genetic heterogeneity and Clericuzio-type Poikiloderma with Neutropenia (CPN) due to mutations in the C16orf57 gene. Mutations in the RECQL4 gene are only observed in two thirds of RTS patients. In this study, 10 patients referred for syndromic poikiloderma and negative for RECQL4 sequencing analysis were investigated for C16orf57 mutations. Two C16orf57 heterozygous nonsense mutations (p.W81X and p.Y89X) were identified in a 5-year-old female child presenting with generalized poikiloderma, dental dysplasia, gingivitis, nail dystrophy, palmoplantar keratoderma and pachyonychia of the great toenails. Previously undetected and silent neutropenia was evidenced after C16orf57 molecular analysis. Neutropenia was absent in the C16orf57-negative patients. This report confirms that neutrophil count should be performed in all patients with poikiloderma to target the C16orf57 gene sequencing analysis, prior to RECQL4 analysis.

Detection of Interstitial Lung Disease in Systemic Sclerosis through Partitioning of Lung Transfer for Carbon Monoxide

Background: Interstitial lung disease (ILD) is a leading cause of death in systemic sclerosis (SSc). Sensitivities and specificities of the current pulmonary function tests (PFTs) for the detection of ILD in SSc are poor. Objective: To determine whether diffusion capacity of the lungs for carbon monoxide (DLCO) partitioned into membrane conductance for CO (DmCO) and alveolar capillary blood volume (Vcap) could provide more sensitive clues to ILD than current PFTs. Methods: DmCO and Vcap were determined in 35 consecutive SSc patients in whom a cardiac and/or pulmonary vascular abnormality had been rejected according to the recommended screening algorithm. ILD was diagnosed with high-resolution computed tomography. Results: Among 35 patients [6 men; median age (first–third quartile) 61.9 years (49.5–67.7)], 22 had no ILD and 13 did. Total lung capacity (TLC), vital capacity and DLCO [percentage of predicted value (%pred)] were lower in patients with ILD [86 (82–103) vs. 106 (98–112), p = 0.01, 96 (88–112) vs. 114 (104–121), p = 0.04, and 67 (59–81) vs. 80 (71–94), p = 0.02, respectively]. DmCO (%pred) and the ratio of DmCO to Vcap were much lower in patients with ILD [54 (48–72) vs. 83 (66–92), p < 0.001, and 0.22 (0.21–0.27) vs. 0.40 (0.35–0.53), p < 0.0001, respectively]. According to receiver operating characteristic analysis, the DmCO:Vcap ratio displayed higher sensitivity and specificity than TLC, vital capacity and DLCO in identifying ILD in our study group (p < 0.01). Conclusions: These results suggest that the partitioning of DLCO might be of interest for identifying ILD in SSc patients.

Sentinel lymph node biopsy in melanoma: Our 8-year clinical experience in a single French institute (2002-2009)

BackgroundSince the introduction of sentinel lymph node biopsy (SLNB), its use as a standard of care for patients with clinically node-negative cutaneous melanoma remains controversial. We wished to evaluate our experience of SLNB for melanoma.MethodsA single center observational cohort of 203 melanoma patients with a primary cutaneous melanoma (tumour thickness > 1 mm) and without clinical evidence of metastasis was investigated from 2002 to 2009. Head and neck melanoma were excluded. SLN was identified following preoperative lymphoscintigraphy and intraoperative gamma probe interrogation.ResultsThe SLN identification rate was 97%. The SLN was tumor positive in 44 patients (22%). Positive SLN was significantly associated with primary tumor thickness and microscopic ulceration. The median follow-up was 39.5 (5–97) months. Disease progression was significantly more frequent in SLN positive patients (32% vs 13%, p = 0.002). Five-year DFS and OS of the entire cohort were 79.6% and 84.6%, respectively, with a statistical significant difference between SLN positive (58.7% and 69.7%) and SLN negative (85% and 90.3%) patients (p = 0.0006 and p = 0.0096 respectively). Postoperative complications after SLNB were observed in 12% of patients.ConclusionOur data confirm previous studies and support the clinical usefulness of SLNB as a reliable and accurate staging method in patients with cutaneous melanoma. However, the benefit of additional CLND in patients with positive SLN remains to be demonstrated.

Carcinome à cellules de Merkel : étude descriptive de 24 cas (1993–2001)

Resume Introduction. – Le carcinome a cellules de Merkel (CCM) est une tumeur rare de mauvais pronostic dont la prise en charge therapeutique optimale reste encore a definir. L’exerese chirurgicale de la tumeur primitive en est le traitement de reference et la realisation d’une radiotherapie ou d’une chimiotherapie adjuvante peut etre discutee. Malades et methodes. – Vingt-quatre cas de CCM recenses sur une periode de 9 ans (dont 17 cas dans le departement du Doubs), ont ete analyses de facon retrospective. Les caracteristiques cliniques et histologiques et la reponse aux differents traitements ont ete evaluees. Resultats. – Nos patients etaient representes par 17 femmes et 7 hommes âges en moyenne de 74,3 ans. Le suivi median des patients etait de 34 mois. L’incidence annuelle pour le departement du Doubs etait de 0,378 pour 100 000 habitants. La localisation cervicocephalique etait predominante (54 %). Lors du diagnostic, 68 % des patients presentaient un stade I et 32 % avaient une localisation ganglionnaire (stade II) ou viscerale (stade III). Pour les 15 patients de stade I, la survie globale a 5 ans etait de 73,85 %. Une recidive locale ou ganglionnaire etait notee dans cinq cas (33 %), bien que deux patients aient ete traites par chirurgie et radiotherapie locale postoperatoire. Pour les stades II et III, la survie globale a 5 ans etait de 51,43 %. Discussion . – Notre serie de 24 patients illustre les caracteristiques cliniques, histologiques et evolutives de cette tumeur rare du sujet âge de mauvais pronostic. La prise en charge therapeutique est discutee.INTRODUCTION Merkel cell carcinoma (MCC) is a rare skin tumor with a highly malignant nature whose appropriate treatment is still debated. Wide surgery is the treatment of choice, but the question concerning protocols for adjuvant radiotherapy or chemotherapy remains open. PATIENTS AND METHODS A retrospective analysis of 24 cases of MCC collected over a period of 9 years was performed, focusing on clinical and histologic features, and response to treatment. RESULTS There were 17 women and 7 men with a mean age of 74.3 years. The median follow up was 34 months. The annual incidence per 100,000 habitants was 0.378. The head and neck localization was predominant (54%). Fifteen (68%) of patients presented with local disease (stage I), and 32% of patients presented with regional node (stage II) or distant metastases (stage III). Patients with stage I had a 5-years overall survival rate of 73,85%. Among them, five patients (33%) developed a local or nodal recurrence, although two patients were initially treated with surgery and local post-operative radiotherapy. Patients with stage II and III demonstrated a 5-year overall survival rate of 51,43%. DISCUSSION Our series illustrates the clinical characteristics of this tumor of the elderly, which is mainly located on head and neck and associated with a poor prognosis. Treatments are discussed.