UK guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016.

The overall objective of the guideline is to provide up-to-date, evidence-based recommendations for the diagnosis and management of the full spectrum of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and SJS-TEN overlap in adults during the acute phase of the disease. The document aims to.

Psoriasis or crusted scabies.

We describe a case of a 67-year-old woman with a 1-year history of nail thickening and a non-itchy erythematous scaly eruption on the fingertips. She was diagnosed with psoriasis and started on methotrexate after having had no response to topical calcipotriol. The diagnosis was reviewed after it was revealed by another consultant that the patient's husband had been attending dermatology clinics for several years with chronic pruritus, which had been repeatedly thought to be due to scabies. Our patient was found to have crusted scabies after a positive skin scraping showed numerous mites. She was treated with topical permethrin, keratolytics and oral ivermectin. We also review the literature on crusted scabies and its management, with recommendations.

Cardiovascular collapse during ethanol sclerotherapy in a pediatric patient.

Ethanol sclerotherapy is a first line management therapy for low flow vascular malformations. It is usually performed under general anesthesia because of the pain associated with ethanol injection. Ethanol sclerotherapy frequently produces minor local complications but may rarely produce catastrophic cardiopulmonary complications. This report describes the cardiovascular collapse associated with an ethanol sclerotherapy procedure in an 11-year- old child. The evidence for ethanol-induced cardiovascular derangements is discussed.

Topical methyl aminolaevulinate photodynamic therapy in patients with basal cell carcinoma prone to complications and poor cosmetic outcome with conventional treatment.

BACKGROUND: Conventional treatment of basal cell carcinoma (BCC) causes morbidity and/or disfigurement in some patients because of the location (e.g. mid-face) and size of the lesion. OBJECTIVES: Following reports that such difficult-to-treat BCC lesions have been treated successfully with topical methyl aminolaevulinate (MAL) photodynamic therapy (PDT), a multicentre study was performed to determine the response of such BCC to MAL-PDT. METHODS: An open, uncontrolled, prospective, multicentre study was conducted comprising patients with superficial and/or nodular BCC who were at risk of complications, poor cosmetic outcome, disfigurement and/or recurrence using conventional therapy. Patients were given one or two cycles within 3 months of topical MAL-PDT, each consisting of two treatments 1 week apart. Tumour response was assessed clinically at 3 months after the last PDT, with histological confirmation of all lesions in clinical remission. The cosmetic outcome was rated. Patients with a BCC in remission will be followed up for 5 years for recurrence, of which the 24-month follow-up is reported here. Ninety-four patients with 123 lesions were enrolled and treated with MAL-PDT at nine European primary care and referral university hospitals. An independent blinded study review board (SRB) retrospectively excluded nine patients and a total of 15 lesions from the efficacy analysis, for not having a difficult-to-treat BCC according to the protocol. RESULTS: The lesion remission rate at 3 months was 92% (45 of 49) for superficial BCC, 87% (45 of 52) for nodular BCC, and 57% (four of seven) for mixed BCC, as assessed by clinical examination, and 85% (40 of 47), 75% (38 of 51), and 43% (three of seven), respectively, as assessed by histological examination and verified by the SRB. At 24 months after treatment, the overall lesion recurrence rate was 18% (12 of 66). The cosmetic outcome was graded as excellent or good by the investigators in 76% of the cases after 3 months follow-up, rising to 85% at 12 months follow-up, and 94% at 24 months follow-up. CONCLUSIONS: Topical MAL-PDT is effective in treating BCC at risk of complications and poor cosmetic outcome using conventional therapy. MAL-PDT preserves the skin and shows favourable cosmetic results.

Sweet's syndrome and polycythaemia rubra vera.

Sweet's syndrome is associated with haematological malignancy, particularly acute myelogenous leukaemia, but there are few reports of its association with polycythaemia rubra vera. We describe an 85-year-old man with polycythaemia rubra vera who developed Sweet's syndrome and review the literature of this association.

Parastomal pyoderma gangrenosum: clinical features and management.

BACKGROUND: The importance of pyoderma gangrenosum (PG) as a cause of ulceration around abdominal stomas is not well recognized. OBJECTIVE: Our purpose was to describe the incidence, clinical and histologic features, disease associations, and possible risk factors for parastomal PG. METHODS: A clinic, run by a dermatologist and two stoma nurses, was created. Five hundred patients approached by postal questionnaire were invited to attend if they had skin problems. In addition, local surgical, dermatologic, and nursing services were invited to refer patients with parastomal skin problems. Cases of parastomal PG were identified, investigated, and treated. RESULTS: The annual incidence of parastomal PG in the questionnaire-based cohort of patients was 0.6% (3 patients). An additional 23 patients with the condition were seen. No consistent hematologic, biochemical, immunologic, microbiological or histologic abnormalities were identified. Local skin damage did not appear to be an important trigger for parastomal PG. The condition is recurrent in one third of cases. Topical tacrolimus (0.3% in carmellose sodium paste) has been effective in 4 patients. CONCLUSION: Parastomal PG is far more common than previous reports would suggest, and it may be associated with diseases other than inflammatory bowel disease.

Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast.

CONTEXT: Zafirlukast is a potent leukotriene antagonist that recently was approved for the treatment of asthma. As use of this drug increases, adverse events that occur at low frequency or in populations not studied in premarketing clinical trials may become evident. OBJECTIVE: To describe a clinical syndrome associated with zafirlukast therapy. DESIGN: Case series. PATIENTS: Eight adults (7 women and 1 man) with steroid-dependent asthma who received zafirlukast. MAIN OUTCOME MEASURES: Development of a clinical syndrome characterized by pulmonary infiltrates, cardiomyopathy, and eosinophilia following the withdrawal of corticosteroid treatment. RESULTS: The clinical syndrome developed while patients were receiving zafirlukast from 3 days to 4 months and from 3 days to 3 months after corticosteroid withdrawal. All 8 patients developed leukocytosis (range, 14.5-27.6 x 10(9)/L) with eosinophilia (range, 0.19-0.71). Six patients had fever (temperature >38.5 degrees C), 7 had muscle pain, 6 had sinusitis, and 6 had biopsy evidence of eosinophilic tissue infiltration. The clinical syndrome improved with discontinuation of zafirlukast treatment and reinitiation of corticosteroid treatment or addition of cyclophosphamide treatment. COMMENT: Development of pulmonary infiltrates, cardiomyopathy, and eosinophilia may have occurred independent of zafirlukast use or may have resulted from an allergic response to this medication. We suspect that these patients may have had a primary eosinophilic infiltrative disorder that had been clinically recognized as asthma, was quelled by steroid treatment, and was unmasked following corticosteroid withdrawal facilitated by zafirlukast.

Effect of the stress of medical residency training on the overnight dexamethasone suppression test.

Forty-two medical residents and interns were studied to evaluate the effect of the stress of medical residency training on-call nights on the hypothalamic-pituitary-adrenal axis. Dexamethasone 1 mg was given at 11:00 p.m. on two separate occasions; a control (off-call) night and on on-call night. Plasma cortisol was measured between 8:00-9:00 a.m. the following morning. The number of patient admissions during the on-call night and the number of hours of sleep during both on-call and control nights were recorded. Plasma cortisol decreased to < 138 nmol/l (< 5 micrograms/dl) in 41 out of 42 (97.6%) of the participants following an on-call day and in 40 out of 42 (95.2%) participants after the control day. The number of hours of sleep during the on-call night was 3.9 +/- 0.29 and during the control night 7.0 +/- 0.11 hours and the number of patient admissions was 3.1 +/- 0.3. In conclusion General Internal Medicine on-call experience does not produce significant alteration on the hypothalamic-pituitary-adrenal axis.

Abuso de cocaína no parenteral en pacientes infectados con el virus de la inmunodeficiencia humana / Non parental cocaine abuse in HIV-1 infected patients

El uso de la cocaína en el Perú ha atravesado por tres etapas. En la época prehispánica mediante la masticación de la hoja y la absorción del alcaloide a través de las mucosas orales. En el comienzo de este siglo por la inhalación intranasal del clorhidrato de cocaína. En los años setenta fumando la pasta de cocaína. Las tres formas de consumo se mantienen hasta hoy, predominando la última entre los sectores populares. La infección por el virus de la inmunodeficiencia humana (VIH-1) hizo su aparición en el Perú hace diez años extendiéndose rápidamente a todas las capas sociales del país, especialmente entre las más pobres. En 163 países infectados con el VIH-1 en el Hospital Dos de Mayo, estudiados mediante un protocolo especial, se ha comprobado que treinta y cuatro (20.8 por ciento) abusaban de la cocaína en forma no parental. Todos los enfermos comenzaron a usar el alcaloide antes de contraer la infección por el VIH-1. Se analiza las caraterísticas demográficas, clínicas y epidemiológicas de estos sujetos. Asimismo, se describe otros factores de riesgo para la diseminación de la infección en este grupo. En conclusión, tanto el abuso de la cocaína como la contaminación con el VIH-1, son hoy graves problemas de salud pública. Se requiere tomar urgentes medidas preventivas y terapéuticas para evitar la extensión de ambas epidemias en nuestro país. Estas acciones correctivas sólo tendrán éxito si se adoptan tanto por las agencias gubernamentales como por los grupos vecinales, particulares y familiares.

Effects of recombinant human IGF-I on glucose and leucine kinetics in men.

To examine the effects of recombinant human (rh) insulin-like growth factor I (IGF-I), insulin, and saline on metabolic parameters, we studied 20 young nonobese healthy men. Euglycemic clamps with 240-min IGF-I infusions at two doses (49 and 33 pmol.kg-1 x min-1, n = 8 and 12 subjects) were performed and compared with hyperinsulinemic-euglycemic clamps (2.25 pmol.kg-1 x min-1, n = 9). Leucine and glucose kinetics were examined with L-[1-13C]leucine and [3-3H]glucose. Glucose rate of appearance (Ra) declined equivalently in the 49 pmol.kg-1.min-1 IGF-I and insulin clamps but remained at basal levels during the 33 pmol.kg-1 x min-1 IGF-I infusions. In contrast, Rd of glucose was increased by 176% in the 49 pmol.kg-1 x min-1 IGF-I and 78% in the 33 pmol.kg-1 x min-1 IGF-I infusions. Furthermore, to prevent hypoglycemia after the termination of both rhIGF-I infusions, it was necessary to infuse glucose for an additional 2-20 h. Ra of leucine was suppressed significantly by both IGF-I and insulin, whereas leucine oxidation was not affected by either hormone. Therefore, the rate of disappearance of leucine expressed as the difference between Ra and oxidation rates was significantly reduced in all clamps. In addition, IGF-I significantly suppressed beta-cell secretion without affecting the other glucoregulatory hormones. In contrast to insulin, IGF-I had no apparent effect on lipolysis, as measured by changes in nonesterified fatty acids.(ABSTRACT TRUNCATED AT 250 WORDS)

Sequential evaluation of islet cell responses to glucose in the transplanted pancreas in humans.

We evaluated the hormonal and metabolic responses of denervated pancreas allografts in nine volunteers 3 to 12 months after the transplant (initial) and again 1 year later (follow-up). Eight of the patients received simultaneous pancreas-kidney transplants. The glucose clamp technique was used to create a square wave of hyperglycemia 5.5 mmol/L above the basal glucose level for 2 hours. A biphasic insulin response was evident in each subject, both initially and at follow-up. The initial plasma insulin response was fourfold higher in patients with pancreas-kidney transplants than in normal volunteers. However, the plasma insulin response of the patients with pancreas-kidney transplants at the follow-up study was more similar to that of the normal controls. The plasma glucagon levels were elevated in follow-up clamp studies. Hepatic glucose production and glucose disposal were similar in both studies. At the follow-up examination only, GLUT4, the major insulin-sensitive glucose transporter, was measured in muscle homogenates by immunoblotting. GLUT4 levels in the patients with pancreas-kidney transplants were only 55% as abundant as in normal volunteers. This may be due, in part, to immunosuppressive therapy or to persistent, albeit reduced, levels of hyperinsulinemia even 2 years after transplantation. We concluded that, despite systemic drainage of the pancreas and immunosuppressive therapy, pancreatic insulin secretion, peripheral insulin levels, and muscle insulin responsiveness are restored toward normal levels approximately 2 years after the transplant.

Clinical comparison of Gentle-Haler Actuator and Aerochamber Spacer for Metered Dose Inhaler (MDI) use by asthmatics.

UNLABELLED: Spacing devices such as the Aerochamber have been shown to improve delivery of medication from MDIs in patients who could not use proper technique with an MDI alone, but the Aerochamber may be inconvenient to carry & use because of its bulkiness. We therefore compared clinical responses of asthmatics using a new, smaller MDI actuator (Gentle-Haler) with no spacer to their responses using a standard MDI actuator & spacer (Aerochamber). METHODS: We treated 30 asthmatic patients in 2 sessions with the beta-agonist albuterol using the above-mentioned devices. Both devices were utilized in each treatment session, with one delivering albuterol & the other generating a placebo. During the second session on a different day, the albuterol & placebo were reversed with respect to the two devices. On each study day, physiologic measurements of FEV1, FVC, FEF25-75, blood pressure, & pulse were obtained at pretreatment (baseline) & after treatment at 15 & 30 minutes & 1, 2, 3, 4, 5 & 6 hours. Analysis of variance & Student's t test were used to compare the ratio of physiologic responses to albuterol delivered with the two devices. RESULTS: Both devices were equally effective in eliciting desirable increases in FEV1, FEF25-75, & FVC. No statistically significant differences (5% significance level) in effectiveness of the albuterol were associated with the use of either device. A very small (< 7%) but statistically significant reduction in diastolic blood pressure (3 of 8 time points) & systolic pressure (1 of 8 time points) was associated with the use of the Gentle-Haler.(ABSTRACT TRUNCATED AT 250 WORDS)

Myocardial energy production and consumption remain balanced during positive inotropic stimulation when coronary flow is restricted to basal rates in rabbit heart.

The effect on myocardial energy balance of increasing oxygen demand without altering basal myocardial perfusion rate was assessed in isolated, isovolumic, retrograde blood perfused rabbit hearts. Myocardial energy requirements were increased with paired stimulation. The capacity of rapid paired stimulation to increase mechanical energy consumption was demonstrated in the presence of increased perfusion with the rate X pressure product and oxygen consumption increasing 86 and 148%, respectively, compared with control values. In contrast, rapid paired stimulation under constant, basal flow conditions did not alter the rate X pressure product, while oxygen extraction and consumption increased only 40% relative to control. Myocardial ATP, creatine-phosphate, and lactate content were identical under control and constant flow-paired stimulation conditions. The results of this study indicate that no detectable energy imbalance was produced by rapid paired stimulation with flow held constant at basal rates. These results suggest that the myocardium does not increase mechanical energy expenditure in response to inotropic or rate stimulation in the presence of restricted flow reserve and are inconsistent with the concept of "demand-induced" or "relative" myocardial ischemia.