[Uncommon cutaneous presentation of visceral Leishmaniasis associated with HIV]. / Manifestations cutanées inhabituelles au cours d'une leishmaniose viscérale associée au VIH.

BACKGROUND: Visceral leishmaniasis is not normally expressed in skin. Herein, we describe the case of an HIV-positive patient who developed two unusual skin manifestations during an episode of visceral leishmaniasis. PATIENTS AND METHODS: A 48-year-old female patient consulted initially for infiltrated purpura of all four limbs. Skin biopsy revealed leukocytoclastic vasculitis with Leishman-Donovan bodies. Laboratory tests showed medullary, splenic, gastric and colic involvement, suggesting systemic disease, and enabling visceral leishmaniasis to be diagnosed. Two years later, despite prolonged treatment, the patient presented maculopapular exanthema, and histology revealed persistent Leishman-Donovan bodies. DISCUSSION: We report herein an association of two rare skin manifestations in an HIV-positive patient with visceral leishmaniasis: infiltrated purpura and maculopapular exanthema. However, the underlying mechanisms remain hypothetical. The initial leukocytoclastic exanthema could be secondary to either polyclonal hypergammaglobulinaemia or to IgA deposits, or possibly to mechanical impairment of blood vessels by the actual parasite. The maculopapular exanthema occurring later raised the possibility of post-Kala-Azar leishmaniasis due to blood-borne dissemination in an anergic subject or perhaps even immune reconstitution inflammatory syndrome.

Epidermolysis bullosa simplex with PLEC mutations: new phenotypes and new mutations.

BACKGROUND: Genetic mutations in the plectin gene (PLEC) cause autosomal recessive forms of epidermolysis bullosa simplex (EBS) associated with either muscular dystrophy (EBS-MD) or pyloric atresia (EBS-PA). Phenotype-genotype analysis has suggested that EBS-MD is due mostly to genetic mutations affecting the central rod domain of plectin, and EBS-PA to mutations outside this domain. OBJECTIVES: This study aimed to describe new phenotypes of patients with EBS-MD and EBS-PA, to identify novel PLEC mutations and to establish genotype-phenotype correlations. METHODS: Seven patients with a suspicion of EBS linked to PLEC mutations were included. A standardized clinical questionnaire was sent to the physicians in charge of each patient. Immunofluorescence studies of skin biopsies followed by molecular analysis of PLEC were performed in all patients. RESULTS: We report the first case of nonlethal EBS-PA improving with age, the first multisystemic involvement in a patient with lethal EBS-PA, and the first patients with EBS-MD with involvement of either the bladder or oesophagus. Eleven novel PLEC mutations are also reported. CONCLUSIONS: Our results confirm that EBS-PA is linked to mutations in the distal exons 1-30 and 32 of PLEC. Long-term survival is possible, with skin improvement, but a delayed onset of MD is probable. While EBS-MD is linked to PLEC mutations in all exons, in most cases one of the mutations affects exon 31. The precocity of MD seems to be linked to the type and localization of the PLEC mutation(s), but no correlation with mucosal involvement has been found.

[Functional impairment and radiologic fasciitis under erlotinib therapy]. / Incapacité motrice et fasciite radiologique sous erlotinib.

BACKGROUND: Targeted molecules are recent and valuable weapons in the management of certain cancers. Among them, erlotinib is an inhibitor of epidermal growth factor receptor approved in non-small lung cancer and pancreatic cancer after failure of first line treatment. Erlotinib is responsible for many cutaneous side effects. We report a case of acute symptomatic fasciitis that has occurred during erlotinib therapy. To our knowledge it is the first case described. CASE REPORT: A 56-year-old man was treated with erlotinib for a metastatic non-small lung adenocarcinoma. Shortly after the treatment by erlotinib was introduced, he had a severe acneiform rash resistant to doxycycline treatment. After a year of treatment, he presented intense pain in the legs with functional impairment. Medical imaging confirmed fasciitis. It regressed along with the rash after using strong topical corticosteroids during ten days. DISCUSSION: Besides bacterial fasciitis, inflammatory and oedematous fasciitis have varied aetiologies. The occurrence of a documented fasciitis during anti EGFR-therapy is original and raises the question of underlying mechanism. We suggest three pathophysiological mechanisms: spreading by contiguity; paraneoplastic fasciitis, or specific lesion of the fascia due to anti-EGFR.

[Severe skin reaction with mucous membrane inflammation during MINE chemotherapy]. / Réaction cutanée sévère avec mucite au cours d'une chimiothérapie de type MINE.

BACKGROUND: MINE chemotherapy is used to treat refractory Hodgkin's disease. Cutaneous adverse effects of MINE regimen are uncommon and chiefly consist of erythema and oedema of the extremities. More recently, a number of cases of panniculitis and subcutaneous inflammatory oedema have been described. OBSERVATION: We report the case of a 17-year-old girl developing acute and painful oedema of the limbs with panniculitis of the trunk. This incident was associated with inflammatory lesions of mucous membrane, in particularly in the genital area and on the tongue. These signs occurred 7 days after initiation of MINE chemotherapy, with no other drugs being introduced. A drug-induced reaction was suspected due to the absence of any other aetiology, particularly infectious disease. The condition gradually improved with symptomatic pain therapy. The patient's chemotherapy was subsequently modified. DISCUSSION: The chronology of the symptoms, spontaneous improvement after the end of treatment, and the absence of other potential causative factors resulted in a hypothesis of a cutaneous adverse reaction to the MINE regimen. The signs could be due to capillary leak syndrome resulting from the toxicity of vinorelbine on endothelial cells. Dermatologists should be aware of these cutaneous adverse effects and of the inflammatory lesions of mucous membrane newly described herein.

[Baboon syndrome due to a polysensitization with corticosteroids]. / Syndrome babouin associé à une polysensibilisation aux corticoïdes.

BACKGROUND: The Baboon syndrome is a form of systemic contact dermatitis. We report the case of a patient who presented a Baboon syndrom following oral medication with betamethasone. CASE REPORT: A 58 year-old man had consulted for eczema involving the groin, the buttocks and the axillae. It was clinically a baboon syndrome related to oral betamethasone (Celestene) treatment, ingested 48 hours earlier. The exploration with a patch-test confirmed the sensitization to betamethasone. This exploration also showed a sensitization to all four classes of corticosteroids. Prolonged withdrawal of all corticosteroids led to the clear improvement of his eczema. DISCUSSION: The baboon syndrome of this patient was the symptom of a systemic eczema due to betamethasone. The patient was sensitized by the percutaneous route. The application of many topical corticosteroids had provoked this polysensitization. It was impossible to consider a safe corticosteroid for this patient.

[Pellagra and panniculitis induced by chronic bacterial colonisation of the small intestine]. / Colonisation bactérienne chronique de l'intestin grêle révélée par un érythème pellagroïde et une panniculite.

INTRODUCTION: Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the progression is fatal. CASE REPORT: A 62 year-old women developed a photoinduced rash, composed of circular and erythematous elements with pustular edges. She also had panniculitis, peripheral neuropathy, depressive mood and diarrhea. Her medical past was marked by epilepsy treated with sodium valproate and hydantoin. Biological exams revealed lowered plasma levels of vitamins PP, B1, B6 and zinc, secondary to intestinal impairment induced by bacteria proliferating in the small intestine. The rash resolved with vitamin PP and zinc supplementation. The bacterial colonisation was improved by long-term, sequential antibiotics. DISCUSSION: We report a rare clinical form of pellagroid rash. The rash was induced by chronic malabsorption resulting from excessive bacterial proliferation in the diverticules of the small intestine. The antiepileptic treatment could have facilitated vitamin PP et zinc deficiency. Panniculitis was related to the bacterial proliferation. We discuss in this paper the relationship between some vitamin deficiencies, their clinical manifestations and the direct role of intestinal bacterial proliferation in the cutaneous manifestations.

Energy and nutrients intake of elderly people living in the Warsaw region, Poland.

The dietary intake of energy, macronutrients, minerals and vitamins was studied in a group of elderly living in urban, suburban, and rural areas of Warsaw region, Poland. The study was conducted in spring 1999 and 298 persons, born between 1919-1924, randomly selected with criteria of age and place of living, were included. Dietary data were based on the 3-day food records. The energy and nutrients intake was calculated on the basis of tables of nutritive value of food products and compared to the Polish RDA's. Daily food rations of the subjects (except for men living in Warsaw) had an incorrect structure as well as insufficient energy content. The highest (i.e. almost 60% or higher) prevalence of intake lower than 2/3 of Polish RDA's was observed for calcium, magnesium, copper, vitamin B2 and vitamin C as well as for iron and vitamin B1 among women. The average intake of fat, cholesterol, and phosphorus was above recommended level. The differences related to area of living were less pronounced for men than for women. Women from suburban area were the most vulnerable group in respect to nutritional inadequacy.

Consumption of food products by the elderly living in different environments of the Warsaw region, Poland.

The aim of the study was to compare qualitatively the intake of food groups by the elderly living in urban and rural areas, with regard to the place of living and sex. Fifty-sex subjects in Warsaw and 142 in villages (born in 1919-24) participated in the study. A 3-day record method was used to collect nutritional data. The subjects living in Warsaw consumed more often milk, meat, fish, fruit and their products, as well as fats and oils. Less than 50% of the elderly living in either rural or urban areas ate eggs and fish. Consumption of fruit and its products was also low in the villages (47%). Meat and meat products were consumed more often by elderly men, whereas milk, fruit and their products were eaten more often by women (a rural area only). Consumption of specific food items also differed between the two analyzed living areas; milk, yoghurt and cheese were eaten more often by the residents of Warsaw, however, the intake of cream was significantly higher in the villages. The intake of red meat and meat products was similar, but poultry was included into the diet of 24% of the elderly in the rural area, significantly less than in Warsaw (34%). It also appeared that butter and mixed fats were more common among the elderly in Warsaw,whereas lard, pork fat and margarine were more popular in the villages. Food choice in the rural area was of poorer quality.

[The use of maternal serum cytokines in the predicting of the efficacy of tocolytic therapy in case of the threat of preterm labor]. / Przydatnosc oznaczen wybranych cytokin w surowicy ciezarnych w przewidywaniu skutecznosci tokolizy w przypadkach zagrazajacego porodu przedwczesnego.

OBJECTIVES: The purpose of our study was to evaluate the use of maternal serum IL-8, IL-6, IFN-gamma levels in the predicting of the efficacy of tocolytic therapy in preterm labor. MATERIALS AND METHODS: We investigated prospectively the group of 47 women in singleton pregnancies with threatened preterm labor in less than 36 weeks gestation and administered tocolytic therapy. RESULTS: In 19 of them tocolysis failed (group II and they delivered premature newborns (the group I--successful tocolysis consisted of remaining 28 women). The incidence of clinical chorioamnionitis, histologic chorioamnionitis and inherited infection of newborns was significantly higher among women refractory to tocolytic therapy (10.2%, 36.8%, 26.3% versus 0%, 3.6%, 0%, respectively, p < 0.05). Maternal serum IL-8, IL-6, IFN-gamma (by means of ELISA technique) and CRP, WBC, ESR levels were measured at the admission to the study. The mean WBC, ESR and the median (range) IFN-gamma (0 (0-7.1) and 0.9 (0-10.4) pg/ml, respectively) didn't differ in both groups. The concentrations of serum IL-8, IL-6, CRP were significantly higher in the group of failed tocolysis (median (range): IL-8: 22.7 (6.3-83.2) vs 3.0 (0-26.0) pg/ml; IL-6: 7.4 (0-21.0) vs 0 (0-11.3) pg/ml; CRP: 1.8 (0.6-7.0) vs 0.6 (0.6-3.9) mg/dl; p < 0.05). Serum IL-8 determinations (definition of abnormal test: > 8 pg/ml) were found the most reliable in the prediction of tocolysis failure with a sensitivity 87.5%, specificity 81.8%, positive predictive value 77.8%, negative predictive value 90% and accuracy 84.2%. Also reliable were IL-6 determinations (IL-6 > 6 pg/ml had a sensitivity 75%, specificity 90.9%, positive predictive value 85.7%, negative predictive value 83.3% and accuracy 84.2%) and CRP determinations (CRP > 1.2 mg/dl had a sensitivity 75%, specificity 81.8%, positive predictive value 75%, negative predictive value 81.8% and accuracy 78.9%). The efficacy of IFN-gamma, WBC and ESR was significantly lower. CONCLUSIONS: Our data revealed that the maternal serum IL-8, IL-6 and CRP determinations are very useful in the predicting of the efficacy of tocolytic therapy in women with threatened preterm labor. The use of IFN-gamma, WBC, ESR was significantly lower.

[Maternal serum cytokines concentrations during normal pregnancy and labor]. / Wyniki oznaczen wybranych cytokin w ciazy niepowiklanej i w porodzie.

OBJECTIVES: The purpose of our study was to determine maternal serum concentrations of IL-8, IL-6, IFN-gamma during normal pregnancy and labor. MATERIALS AND METHODS: Maternal serum IL-8, IL-6 and IFN-gamma levels were measured by means of ELISA technique in 41 healthy pregnant women in 22-42 week gestation and 15 healthy women in labor at term. All newborns and afterbirths had no signs of infection. RESULTS: IL-8 values for pregnant women ranged from 1.98 to 35.2 pg/ml with the median value 10.24 pg/ml, and the 95th percentile 24.5 pg/ml. IL-8 values for women in labor at term ranged from 3.96 to 54.8 pg/ml with the median 10.4 pg/ml. No statistically significant changes in serum IL-8 concentration were observed during pregnancy or in labor. Serum IL-6 concentrations in pregnant women ranged from 0 to 21.7 pg/ml with the median value 0 pg/ml, and the 95th percentile 15.5 pg/ml. Serum IL-6 concentrations in women in labor at term were significantly higher (p < 0.05): ranged from 0 to 39.2 pg/ml with the median 10.1 pg/ml and 95-th percentile 33.5 pg/ml. Maternal serum IFN-gamma concentrations in pregnant women ranged from 0 to 9.8 pg/ml with the median value 3.9 pg/ml, the 95th percentile 9.2 pg/ml and didn't differ during labor at term: range from 0 to 14.5 pg/ml, median 1.9 pg/ml. CONCLUSIONS: Our data revealed that maternal serum IL-8 concentrations didn't changed during the course of pregnancy and in labor. Women in labor had significantly elevated serum IL-6 concentrations compared to those in pregnancy. We didn't observed such changes in serum IFN-gamma levels.

[Infection with parvovirus B19 in pregnant women]. / Infekcje parvowirusem B19 u kobiet ciezarnych.

The viral infection caused by Parvovirus B19 which occurs at pregnant women may be reason of many different kinds of complications during pregnancy. Until this time it is not known the frequency of the Parvovirus infections at pregnant women in Poland. We have based our studies on a group of 78 pregnant women with symptoms of a abortion, a premature imminent labour, premature labor and intrauterine death of foetus. In 10 cases (12.8%) we have confirmed a presence of antibodies IgM class antiparvovirus B19 at patients serum. It seems that the Parvovirus infection is one of most often reasons of unsuccessful pregnant.

[Maternal serum cytokines in labor, pregnancy and chorioamnionitis]. / Wyniki oznaczen wybranych cytokin w surowicy ciezarnych oraz rodzacych przedwczesnie i o czasie.

UNLABELLED: The purpose of our study was to compare maternal serum levels of interleukin-6, interleukin-8, tumor necrosis factor-alpha and interferon-gamma in gravidities, during spontaneous term and preterm labor and their relation to histologic chorioamnionitis. METHODS: We investigated 61 women: 10 in preterm labor, 36 in term labor and 15 healthy pregnant nonlabouring controls. Venous bloods for cytokines determinations were obtained during the first stage of labor and during routine screening tests. Titers of cytokines were measured by means of ELISA technique. All births after preterm deliveries were examined to establish histologic chorioamnionitis. RESULTS: Serum levels of IL-6 and IL-8 were significantly elevated both in term (mean: IL-6: 17.5 +/- 58 pg/ml; IL-8: 148 +/- 215 pg/ml) and preterm labor (IL-6: 23 +/- 44 pg/ml; IL-8: 332 +/- 389 pg/ml) when compared to nonlabouring gravidities (IL-6: 5 +/- 7 pg/ml; IL-8: 14 +/- 11 pg/ml). IL-6 and IL-8 titers were statistically similar in term and preterm labors and in patients with and without histologic chorioamnionitis. TNF-alpha and IFN-gamma were not statistically analyzed because only a few patients had detectable serum levels of these cytokines. CONCLUSION: Serum levels of IL-6 and IL-8 in both: term and preterm labor are elevated in comparison to nonlabouring gravidities. The elevated levels of these cytokines are not connected with coexisting chorioamnionitis.

[Serum cytokines in patients with ovarian cancer and benign ovarian cysts]. / Wyniki oznaczen wybranych cytokin w surowicy chorych na raka jajnika lub lagodne torbiele jajników.

UNLABELLED: The aim of our study was to evaluate serum interleukin-6 (IL-6), interleukin-8 (IL-8), tumor necrosis factor-alpha (TNF-alpha) and interferon-gamma (IFN-gamma) as tumor markers--study based on the data about tumor cytokines production and tumor-host interactions. METHODS: We investigated 48 women: 17 with ovarian cancer untreated before, 16 with benign ovarian cysts and 15 healthy controls. Venous blood for cytokines determinations were obtained before operations and during routine screening tests. Titers of cytokines were measured by means of ELISA technique. RESULTS: In the control group the upper limit of normal IL-6 titers (95th percentile) was 5.5 pg/ml; the mean IL-8 concentration was 9.6 +/- 15.1 pg/ml and the upper limit of normal was 37 pg/ml; serum TNF-alpha and IFN-gamma were not detectable. In patients with benign ovarian cysts the levels of all investigated cytokines didn't differ significantly from healthy controls. Women with ovarian cancer had significantly higher serum IL-8 levels (mean: 290.5 +/- 351 pg/ml) than healthy controls or women with benign ovarian cysts; 88% of them had IL-8 titers above the normal. The IL-6 titers in ovarian cancer were also higher but didn't reach statistical significance, 53% of them had IL-8 titers above the normal. TNF-alpha and IFN-gamma levels in ovarian cancer were similar to patients with benign ovarian cysts. CONCLUSION: Serum IL-8 levels in patients with ovarian cancer were significantly higher when compared to healthy controls and benign ovarian cysts and in almost 90% of ovarian cancers the titers of IL-8 were increased. Additionally, 53% of women with ovarian cancer had elevated serum IL-6 levels.

[Methods of nutrient intake and nutritional status of the elderly in Poland on the basis of the literature from the years 1980-1996]. / Sposób zywienia oraz stan odzywienia ludzi starszych w Polsce w swietle danych z pismiennictwa z lat 1980-1996.

According to papers published in the years 1980-1996 there were many faults in nutritional habits of the elderly in Poland, which can affect nutritional and health status. Low consumption of calcium, vitamins C, A, and B group was identified most often while the intake of fats was too high in comparison to Polish recommended daily intake. Moreover iron and protein intake among women were also too low. The analysis of food products intake indicated that above mentioned inadequacy in nutrient intake was the result of low consumption of milk and milk products, fruits and vegetables containing vitamin C and beta- carotene as well as grain products and potatoes and high consumption of products from the group other fats. The observed differences between intake and nutritional recommendation were confirmed by the indices of nutritional status. BMI indicating overweight or obesity was observed for many subjects under study, more frequently among women than among man. Biochemical analysis showed risk of deficiency of some vitamins.

[Erythropoietin in the pathogenesis of anemia in patients with multiple myeloma]. / Erytropoetyna w patogenezie niedokrwistosci u chorych na szpiczaka mnogiego.

UNLABELLED: Anemia is a common symptom in multiple myeloma (MM) patients but the pathogenesis of it is still unknown. The aim of the study was to explain the causes of anemia in MM patients. Peripheral blood count, bone marrow aspirate, iron and ferritin level, serum erythropoietin (EPO) level, T cell subsets and in vitro CFU-E count were analyzed in the group od 31 MM patients. Erythropoietin and iron deficiency in the study group were not observed. EPO serum level was not significantly different in patients with multiple myeloma and in comparison to patients with sideroblastic anemia with solid tumors. Absolute CD8 T lymphocyte count was not significantly increased in the study group. CFU-E colonies count in vitro was not decreased in these patients. CONCLUSIONS: In the study group of the MM patients anemia probably does not depend on EPO production. Diminished proliferative response of erythropoietic cells on normal serum level of EPO and abnormal iron utilisation probably occur in these patients. Replacement of normal erythropoiesis by tumor plasma cells is probably not decisive.