Biomedical subjects

E Puzenat

Publications and source records attributed to E Puzenat.

12 recordsLinked to original sources

PubMed2006

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. OBJECTIVE: To study epidemiological, immunohistochemical and clinical features, delay in diagnosis, type of treatment and outcome of DFSP from 1982 to 2002. METHODS: Using data from the population-based cancer registry, 66 patients with pathologically proved DFSP were included (fibrosarcomatous DFSP were excluded). Each patient lived in one of the four departments of Franche-Comté (overall population of 1 million people) at the time of diagnosis. The main data sources came from public and private pathology laboratories and medical records. The rules of the International Agency for Research on Cancer were applied. RESULTS: The estimated incidence of DFSP in Franche-Comté was about three new cases per 1 million people per year. Male patients were affected 1.2 times as often as female patients were. The trunk (45%) followed by the proximal extremities (38%) were the most frequent locations. DFSP occurred mainly in young adults between 20 and 39 years of age. Mean age at diagnosis was 43 years, and the mean delay in diagnosis was 10.08 years. Our 66 patients initially underwent a radical local excision. Among them, 27% experienced one or more local recurrences during 9.6 years of follow-up. There was one regional lymph node recurrence without visceral metastases. These recurrences were significantly related to the initial peripheral resection margins. We observed a local recurrence rate of 47% for margins less than 3 cm, vs. only 7% for margins ranging from 3 to 5 cm [P=0.004; OR=0.229 (95%, CI=0.103-0.510)]. The mean time to a first local recurrence was 2.65 years. Nevertheless, there was no death due to the DFSP course at the end of the follow-up, and the final outcome was favourable. CONCLUSION: Our study emphasizes the importance of wide local excision with margins of at least 3 cm in order to prevent local recurrence. However, the recent development of inhibitors of signal transduction by the PDGFB pathway should soon modify the surgical strategy, which is often too mutilating.

Adolescent↗

PubMed2005

[Incomplete expression of PHACE (S) syndrome: facial hemangioma associated with absence of the internal carotid artery].

INTRODUCTION: Hemangiomas are the most common benign tumors in childhood. In rare cases, they can be associated with dysmorphic malformations. The acronym, the PHACE (S) syndrome, was recently described by Frieden et al. in 1996 as a large facial or cervical Hemangioma, associated with one or more of the following systemic abnormalities including:Posterior fossa malformation, Arterial abnormalities, Coarctation of the aorta and/or cardiac defects, Eye abnormalities and Sternal clefts. CASE REPORT: A 2 year-old girl presented with a large left hemifacial hemangioma. The rest of the clinical examination was normal. Initially, simple clinical surveillance was scheduled. The outcome was good with almost complete regression of the hemangioma by the age of 8. However, there were remains to the left of the upper lip and plastic surgery was scheduled. Pre-operative conventional arteriography revealed the complete, asymptomatic, absence of the ipsilateral internal carotid artery. Cerebral MRI and cardiac ultrasonography were normal. In the absence of somatic manifestations, regular clinical surveillance was decided on. DISCUSSION: Large facial or cervical hemagiomas can be associated with one or more systemic abnormalities described by the PHACE (S) acronym. Its prevalence is unknown, but is shows marked female preponderance. Among the systemic abnormalities, neurological and cardiac malformations predominate. Hence the PHACE (S) syndrome must be recognized. Moreover, in patients presenting with large facial or cervical hemangioma, the following examinations should be performed: neurological examination and cerebral MRI to rule out abnormality of the posterior fossa, completed by a sequence of angio-MRI in the search for cerebral artery malformations; cardiovascular exploration, completed by echocardiography in the case of doubt and examination of the eyes and sternum. Lastly, the enhanced risk of laryngeal sub-glottis hemangioma should be kept in mind.

Abnormalities, Multiple↗

PubMed2005

Evaluation of a novel 308-nm monochromatic excimer light delivery system in dermatology: a pilot study in different chronic localized dermatoses.

BACKGROUND: Recently, units have been developed that are capable of delivering large fluences of narrowband ultraviolet (UV) B selectively to cutaneous lesions within a reasonable time. OBJECTIVES: To analyse the efficacy of a novel nonlaser 308-nm monochromatic excimer light (MEL) delivery system in various dermatoses usually treated by narrowband UVB phototherapy. METHODS: Fifty-four patients with chronic and resistant localized dermatoses were enrolled in a prospective study: 17 with palmoplantar pustular psoriasis, seven with plaque-type psoriasis, four with nail psoriasis, eight with chronic atopic dermatitis of the hands, 10 with chronic nonatopic dermatitis of the hands and eight with alopecia areata. The 308-nm xenon chloride MEL delivery system (Excilite; DEKA, Florence, Italy) was used to produce an average incident dose rate of 50 mW cm(-2) at a tube-to-skin distance of 15 cm and with a maximum irradiating area of 512 cm2. The initial dose was based on multiples of a predetermined minimal erythema dose (MED), and subsequent doses were based on the response to treatment. Treatments were scheduled weekly for a maximum of 10 weeks. Clinical responses were evaluated using photographic documentation and (except for alopecia areata) clinical score. RESULTS: The MED ranged from 250 to 350 mJ cm(-2) (mean +/- SD 318.2 +/- 28.4). MEL at 308 nm was the most effective for palmoplantar pustular psoriasis with a mean improvement of 79% after a mean of 5.3 treatments and a mean dose of 11.8 MED per treatment. Plaque-type psoriasis was significantly less sensitive to treatment and nail psoriasis demonstrated no benefit from treatment. Chronic palmar atopic dermatitis was cleared in two patients and the mean improvement was 54% as compared with 46% in patients with chronic nonatopic dermatitis of the hands. Four complete regrowths among the eight patients with alopecia were observed after a mean of 5.1 treatments. The percentages of improvement had significantly decreased at the 6-month visit, and only four patients (24%) with palmoplantar pustular psoriasis still demonstrated a significant improvement. Common side-effects included intense erythema and, more rarely, blisters, but these were well tolerated. CONCLUSIONS: Our preliminary results confirm the efficacy of this novel 308-nm MEL delivery system, which appears to be effective and safe for palmoplantar pustular psoriasis. To a lesser extent, plaque-type psoriasis, chronic atopic and nonatopic dermatitis of the hands and alopecia may also benefit from this treatment.

Adult↗

PubMed2005

Circulating vascular endothelial growth factor in cutaneous malignant melanoma.

BACKGROUND: Angiogenesis has been reported as a parameter of potential prognostic value in solid tumours, as it may facilitate tumour growth and metastasis. One of the most important growth factors involved in angiogenesis is vascular endothelial growth factor (VEGF). OBJECTIVES: To determine the predictive value of circulating VEGF levels in a cohort of patients with melanoma. METHODS: In a prospective cohort study, 324 patients with cutaneous melanoma at different clinical stages were investigated over 2 years (2002-04). VEGF was measured in plasma using enzyme-linked immunosorbent assay. Two hundred and eight patients were able to be followed up for progression of their disease and for blood sample collection (mean +/- SD follow-up 13.4 +/- 0.8 months). Data were compared with the extent of the disease and the clinical course. RESULTS: A significant increase in plasma VEGF levels was found in patients with melanoma compared with healthy controls, with statistically significant differences between patients in stages I, II and III vs. those in stage IV, but not between patients in stages I, II and III. When considering the 237 patients in stages I and II, no statistical correlation was found between plasma VEGF levels and tumour thickness. Baseline plasma VEGF levels were not significantly higher in patients who relapsed compared with nonprogressing patients. Among the 35 patients (two stage I, eight stage II and 25 stage III) who experienced a progression during follow-up, an increase in plasma VEGF level to > 100 pg mL(-1) was found in 20 (sensitivity 57.1%), while 38 of the 173 remaining nonprogressing patients demonstrated an increase in VEGF level, indicating a specificity of 78%. In addition, an increase in plasma VEGF level was found in 58 patients during follow-up, of whom 20 showed evidence of progression, indicating a positive predictive value of 34.5%. However, among the 150 remaining patients who did not demonstrate any increase in plasma VEGF level during follow-up, only 15 experienced a progression, indicating a negative predictive value of 90%. CONCLUSIONS: Our data confirm that blood VEGF levels are significantly increased in patients with melanoma and, more interestingly, that the absence of plasma VEGF level increase during follow-up appears to be associated with remission.

Disease Progression↗

PubMed2004

[Pruritus sine materia: a prospective study of 95 patients].

PURPOSE: To describe the nature and the frequency of systemic diseases responsible for the pruritus sine materia. Value of this sign as a marker of malignancy. METHODS: Prospective study undertaken over five years (1996-2001). INCLUSION CRITERIA: generalized aspect of the pruritus and absence of specific primitive cutaneous lesions of an itching dermatosis. Parameters taken from the data of the anamnesis and the physical examination. Standard biologic and morphologic investigations were done. RESULTS: Ninety-five patients included (54 men, 41 women) of 55.5 years average age +/-18.1. In 24 patients, traditional hospitalisation with one average duration of eight days stay +/-3.15 was necessary. In 38 cases (40%), a systemic cause was found. The main conditions were: toxocariasis (8 cases), hematologic diseases (7 cases), chronic renal failure (6 cases), hypothyroidism (5 cases) and iron deficiency (5 cases). A neoplasm was found in eight cases (8,42%): seven hematologic malignancy (3 myeloma, 2 Hodgkin's diseases, 2 myeloproliferative syndromes) and one solid cancer (pulmonary adenocarcinoma). CONCLUSION: A systemic aetiology was observed in 38 cases (40%). The toxocariasis an underestimated disease comes at the first place. The pruritus sine materia can hide an hematologic malignancy.

Adult↗

PubMed2004

[Ecthyma gangrenosum caused by Pseudomonas stutzeri with bacteraemia and systemic vascularitis].

INTRODUCTION: Ecthyma gangrenosum is a cutaneous manifestation of Pseudomonas infections. This condition may be associated with bacteraemia but can also occur in the absence of bacteraemia. EXEGESIS: The authors report the case of a 66-year-old woman presented with necrotic ulcerations on the face associated with fever, arthralgia, myalgia, fatigue and neutropenia. Blood cultures and skin cultures were positive for Pseudomonas stutzeri. Her condition improved under appropriate antibiotic therapy. However, the patient further developed clinical and biological symptoms of systemic vasculitis and mixed cryoglubulinemia. Complete healing was finally obtained after a course of corticosteroids. CONCLUSION: Ecthyma gangrenosum should be suspected in people who have typical clinical presentation. This disease could sometimes be associated with systemic vasculitis.

Bacteremia↗

PubMed2003

[Merkel cell carcinoma: descriptive study of 24 cases (1993-2001)].

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.

Aged↗

PubMed2003

Spectrum of cross-photosensitization in 18 consecutive patients with contact photoallergy to ketoprofen: associated photoallergies to non-benzophenone-containing molecules.

Contact photoallergy to ketoprofen gels has been widely reported, and cross-sensitivity reactions with other compounds, such as tiaprofenic acid, fenofibrate and benzophenones, are well known. However, positive photopatch tests to other different non-benzophenone-related compounds have recently been observed. We report the results of photopatch testing in patients with contact photoallergy to ketoprofen and discuss the spectrum of cross-sensitization to ketoprofen. 18 consecutive patients with a history of photocontact dermatitis from ketoprofen were investigated. Patch and photopatch tests were performed. As expected, we observed positive photopatch tests to Ketum* gel and ketoprofen 2.5% in petrolatum in all patients (100%). However, it was remarkable to note positive photopatch tests to other unexpected and non-relevant allergens, including fentichlor (67%), tetrachlorosalicylanilide (28%), triclosan (17%), tribromsalan (11%) and bithionol (11%), with no clinical relevance. Interestingly, these agents belong to the family of halogenated salicylanilides and related compounds, which have been forbidden in Europe since the 1970s. Our results raise the question of hyper-photosusceptibility to non-relevant allergens induced by photosensitivity to ketoprofen. The mechanism may involve the high photoreactivity induced by the association of a benzene ring with an oxygen group.

Adult↗

PubMed2002

[Erythrodermia].

Explore the source record for details and available documents.

Adult↗

PubMed2001

Genital squamous cell carcinoma in men treated by photochemotherapy. A cancer registry-based study from 1978 to 1998.

BACKGROUND: One single report from the U.S. 16-centre-trial indicated that psoralen and ultraviolet A radiation (PUVA) therapy may induce an increased risk of genital tumours in men, and protection of the genital area is, therefore, recommended. OBJECTIVES: To evaluate the relevance of this risk in routine clinical practice. METHODS: Two groups of patients were included in a 1978-98 retrospective study. Case records of men with genital squamous cell carcinoma (SCC) identified from the Cancer Registry of the Doubs area of France were examined for a history of PUVA therapy, topical tar treatment, psoriasis, human papillomavirus infection or genital dermatitis. In addition, all the dermatologists of the Doubs area (in public and private practice) using PUVA therapy were asked to provide information on the number of patients having received PUVA therapy and whether the genital area was exposed during treatment. RESULTS: Between 1978 and 1998, among the 48 men who had developed a genital SCC in the Doubs area, only one had a history of intensive PUVA therapy. About 150,000 treatments with PUVA therapy had been performed by 15 dermatologists in the Doubs area for 5400 patients since 1978. No case of genital SCC had been reported, despite the fact that the genital area had not been protected during UVA exposure. CONCLUSIONS: Although retrospective, our study demonstrates that the occurrence of genital SCC in men treated with PUVA therapy is a very rare event in common dermatological practice.

Adult↗

PubMed2000

[Subcutaneous fat necrosis of the newborn with hypercalcemia].

INTRODUCTION: Hypercalcemia associated with subcutaneous fat necrosis of the newborn is a well known but rare event. CASE REPORT: A newborn with a history of cesarean section, fetomaternal infection, neurological and respiratory distress was admitted with anorexia, adynamia, vomiting, polyuria and polydipsia at the age of 37 days. Physical examination showed red and violaceous infiltrated skin lesions. Skin biopsy revealed focal areas of fat necrosis with crystal-like structures. Calcium and 1,25 (OH(2) ) vitamin D serum levels were increased. Diagnostic of subcutaneous fat necrosis of the newborn with symptomatic hypercalcemia was made. Evolution was favorable after treatment including furosemide, prednisone and a diet low in calcium and vitamin D. DISCUSSION: Hypercalcemia must be detected in infants with subcutaneous fat necrosis. This major complication may have fatal outcome. Treatment of hypercalcemia includes dietary changes associated with classic treatment of hypercalcemia. Routine evaluation of serum calcium level is essential.

Fat Necrosis↗

PubMedDate not supplied

[Iatrogenic acrodermatitis enteropathica-like syndrome in leucinosis].

INTRODUCTION: Leucinosis (maple syrup urine disease) is a metabolic disorder caused by an enzymatic deficiency involved in the degradative pathways of the three branched-chain amino acids. We report an observation of acrodermatitis enteropathica-like syndrome induced by essential amino acid deficiency in a child with leucinosis. CASE REPORT: A child with leucinosis was referred to our hospital for exfoliative dermatitis of the perioral and anogenital regions associated with diarrhea and pancytopenia. The diagnosis of iatrogenic acrodermatitis enteropathica-like syndrome was confirmed after screening showing isoleucine deficiency. Rapid response was observed after adequate isoleucine supplementation. DISCUSSION: The acrodermatitis enteropathica-like eruption in our patient was due to an iatrogenic amino acid nutritional imbalance. Our observation underlines the risk of using a branched-chain amino acid-free formula without adequate supplementation of deficient amino acids. In addition, dietary insufficiency of isoleucine, associated with the treatment of organic aciduria should be added to the causes of acrodermatitis enteropathica-like syndrome.

Acrodermatitis↗