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Hutt Valley District Health Board, Hutt Hospital, High Street, Lower Hutt, Private Bag 31-907 Lower Hutt, 5040 New Zealand.
The Stevens-Johnson syndrome (SJS) classically involves a rash, conjunctivitis and mucositis. We describe the case of a young adult male with isolated mucositis and conjunctivitis . Previous rare reports of severe SJS like syndromes without a rash are confined to children, usually with mycoplasma pnemoniae infection. Terminology for this syndrome includes -"Stevens-Johnson Syndrome without skin lesions", or "Atypical Stevens - Johnson Syndrome". This case highlights the importance of maintaining an open mind when a "full house" of clinical features is absent. It also illustrates the use of a rapid electronic literature review as a clinical tool. The importance of updating records when a drug has been cleared of causing harm is highlighted.

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Weil, Gotshal & Manges LLP, New York, New York, USA.
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Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
OBJECTIVE To determine the occurrence of fat-soluble vitamin deficiencies and to identify clinical factors that may predict vitamin deficiency in patients with primary biliary cirrhosis (PBC). METHODS Review of our data from a randomized, placebo-controlled trial that evaluated the efficacy of UDCA in 180 patients with PBC. We use the first available measurements of vitamin levels in each study participant. Vitamin levels for A, D, and E were measured in serum. The prothrombin time (PT) was used as a surrogate marker for vitamin K. RESULTS The proportion of patients with fat-soluble vitamin deficiencies in the treatment and placebo groups was similar and the data sets were combined. The proportion with vitamin A, D, E or K deficiency was 33.5%, 13.2%, 1.9%, and 7.8%, respectively. In multivariate analysis, the Mayo risk score, advanced histological stage, and total cholesterol were independently associated with vitamin A deficiency whereas serum albumin levels was independently associated with vitamin D deficiency. No factors were associated with vitamin E or K deficiency in multivariate analysis owing to the few vitamin E and K deficient patients. Factors predictive of vitamin K deficiency by univariate analysis included Mayo risk score, advanced histological stage, HDL, total bilirubin, AST, and albumin. The cut-off value of the Mayo risk score with the highest sensitivity and specificity for vitamin A deficiency was 5.0. CONCLUSION Other than deficiency of vitamin A, deficiency of fat-soluble vitamins occurs uncommonly in patients with PBC. A Mayo risk score > or = 5 helps in selecting patients with PBC for surveillance for vitamin A deficiency.
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OBJECTIVE: To measure well-being and treatment satisfaction and their correlates in older people with diabetes. RESEARCH DESIGN AND METHODS: A postal survey was conducted of 1,000 diabetic patients aged > or = 60 years, representing 56% of the resident older diabetic population in an inner-city health district with a largely indigenous population of 230,000 people and a widely varied socioeconomic mix. Well-being and treatment satisfaction were measured with diabetes-specific instruments and correlated with patient data held in a central register. RESULTS: There was an 81% response. The general well-being scores (median [interquartile range]) for patients on diet alone, tablets, and insulin were 54 (44-60), 53 (42-61), and 48 (35-56)(P < 0.001 comparing insulin with diet and tablets) compared with a scale maximum of 66. Treatment satisfaction scores were 35 (31-36), 35 (32-36), and 34 (30-36)(P < 0.001 comparing insulin with diet and tablets), scale maximum 36. Mean HbA1c concentrations were 5.0 +/- 1.4%(for patients on diet alone), 5.8 +/- 1.6%(tablets), and 6.6 +/- 1.7%(insulin)(P < 0.001 for each difference). Neither well-being nor treatment satisfaction correlated with HbA1c. Insulin-treated patients were younger and had been diabetic longer than non-insulin-treated patients; their well-being remained slightly, but significantly, lower when adjusted for age, sex, BMI, and diabetes duration, but treatment satisfaction was no longer significantly different. Women had lower well-being than men. CONCLUSIONS: It has proved possible to measure well-being and treatment satisfaction in a large community-based samples of older people with diabetes. At the level of glycemic control in this population, neither parameter correlated with HbA1c. The lower well-being in insulin-treated patients remained significant in multivariate analysis.
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Department of Food Animal and Equine Medicine, College of Veterinary Medicine, North Carolina State University, Raleigh 27606, USA.
Northern mockingbirds (Mimus polyglottos) and blue jays (Cyanocitta cristata) in a Florida (USA) wildlife care facility developed clinical signs and gross lesions suggestive of the ongoing outbreak of Mycoplasma gallisepticum (MG) conjunctivitis in house finches (Carpodacus mexicanus) and American goldfinches (Carduelis tristis). Mycoplasmal organisms were cultured from conjunctival/corneal swabs of birds with sinusitis, conjunctivitis, and/or epiphora. All of the isolates tested were identified as Mycoplasma sturni by indirect immunofluorescence. Mycoplasma sturni as well as MG should be considered in the differential diagnosis of songbirds with conjunctivitis.
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Division of Area General Internal Medicine, Mayo Clinic, Rochester, Minn, USA.
BACKGROUND Although oral and inhaled glucocorticoid therapy may impair growth in children with asthma, the effect of glucocorticoid therapy and asthma on attained adult height has not been extensively studied in representative children in the community. OBJECTIVES The study was designed to compare the attained adult height of children with asthma with the attained adult height of nonasthmatic children and to compare the attained adult height of asthmatic children treated with glucocorticoids with the attained adult height of asthmatic children who did not receive glucocorticoids. METHODS Residents of Rochester, Minnesota, with onset of asthma from 1964 to 1987 and age- and sex-matched non-asthmatic residents of Rochester were studied. Glucocorticoid exposure was assessed from medical records. The mean of 5 stadiometer measurements of adult height, adjusted for sex and parental height, was analyzed. RESULTS One hundred fifty-three patients with asthma (mean age at onset, 6.1 +/- 4.8 years) and 153 age- and sex-matched nonasthmatic subjects were studied. Adult height of patients with asthma (mean age at measurement, 25.7 +/- 5.2 years) was not significantly different from the adult height of non-asthmatic subjects; the overall difference, adjusted for mid-parental height, was -0.20 cm (95% confidence interval from -0.27 to 1.64). The adult height of asthmatic children treated with glucocorticoids was not significantly different from the adult height of patients with asthma not treated with glucocorticoids; the difference after adjusting for mid-parental height was -0.2 cm (95% confidence interval from -0.1 to 0.6). CONCLUSIONS We conclude that the attained adult height of patients with asthma is not different from the adult height of age- and sex-matched nonasthmatic subjects and that the attained adult height of asthmatic children treated with glucocorticoids is not significantly different from the adult height of children not treated with glucocorticoids.
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Department of Food Animal and Equine Medicine, College of Veterinary Medicine, North Carolina State University, Raleigh 27606, USA.
In separate trials, layer pullets were vaccinated with Mycoplasma gallisepticum (MG) strain 6/85 or strain ts-11 commercially produced live vaccines. For a 15-wk postvaccination (PV) period, vaccinates were commingled with unvaccinated pullets and were in indirect contact with sentinel groups of pullets, broiler breeders, turkey breeders, or meat turkeys in adjoining pens. Infectivity and transmissibility of vaccine strains were determined by tracheal culture and serology at 1 wk followed by 3-wk intervals PV. Strain 6/85 was recovered from 0%-20% of vaccinates, but not from commingled pullets or sentinel birds. Strain ts-11 was recovered from 60%-90% of vaccinates and 0%-40% of commingled pullets but not from any of the sentinel birds. No birds in the 6/85 vaccine trial tested positive for MG antibodies by serology. MG enzyme-linked immunosorbent assays detected positive responses in ts-11 vaccinates (range = 10%-70%) at 42, 63, 84, and 105 days PV, and commingled pullets (10%) at 84 and 105 days PV. MG serum plate agglutination tests detected positive responses in 90% and 20% of ts-11 vaccinates at 42 and 105 days PV, respectively, and commingled pullets (10%) at day 42 PV. Clinical signs, morbidity, or mortality suggestive of pathogenic MG infection were not observed in any bird during either trial, and no gross lesions were observed at necropsy. Random amplified polymorphic DNA analysis was capable of distinguishing each of the vaccinal strains 6/85 and ts-11 from each other by their distinct DNA banding patterns.
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College of Veterinary Medicine, North Carolina State University, Raleigh 27606, USA.
Membrane proteins of Mycoplasma gallisepticum (MG) strain R were extracted with the detergent Mega-10 and incorporated into immunostimulating complexes (ISCOMs). A membrane protein of approximately 64 kD (p64) molecular weight was a major component of MG ISCOMs. Six-week-old specific-pathogen-free leghorn chickens were inoculated by various routes (subcutaneous; combined intranasal and eyedrop; and combined subcutaneous, intranasal, and eyedrop) with 10 micrograms MG proteins in ISCOMs, or inoculated subcutaneously with 10 micrograms MG proteins in Freund's adjuvant. Subcutaneous inoculation of MG ISCOMs, or MG Freund's adjuvant resulted in higher sero-positive rates (detected by enzyme-linked immunosorbent assay) in serum and respiratory tract washings, compared to combined routes of MG ISCOM inoculation. In chickens inoculated subcutaneously with MG ISCOMs antibodies were first detected at 31 days postinoculation (PI) and the sero-positive rate peaked at 56 days PI. Sero-positive rates started to decline at day 64 PI. In the Freund's adjuvant group, MG antibodies were first detected at day 21 PI, and the sero-positive rate peaked at day 39 PI and did not decline. MG antibodies were detected by ELISA in upper respiratory tract and tracheal washes from chickens inoculated subcutaneously with MG ISCOMs and MG Freund's adjuvant. Immunoblots to MG strain R whole cell proteins showed that respiratory tract washings and sera from chickens inoculated subcutaneously with MG ISCOMs contained immunoglobulins to MG proteins, with a prominent reaction to p64.
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Mayo Clinic, Rochester, MN 55905, USA.
Cause of death among 917 of the 959 subjects in a population-based incidence cohort with Alzheimer's disease (AD) during the years 1960 to 1984 was compared to that of an age- and gender-matched control group (n = 703). In general, a summary of the diagnostic codes entered anywhere on a death certificate suggests that control subjects had more cardiovascular disease and neoplasms than did patients, while patients more often had a diagnostic code of bronchitis/pneumonia (P < 0.01). By logistic regression, this difference remained statistically significant after adjusting for age and gender.
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Department of Food Animal and Equine Medicine, College of Veterinary Medicine, North Carolina State University, Raleigh 27606, USA.
An epornitic of conjunctivitis in free-flying house finches (Carpodacus mexicanus) occurred in several mid-Atlantic and eastern states of the USA in 1994. Clinical signs and gross lesions ranged from mild to severe unilateral or bilateral conjunctival swelling with serous to mucopurulent drainage and nasal exudate. Microscopic lesions consisted of chronic lymphoplasmacytic conjunctivitis, rhinitis, and sinusitis. Notably slow-growing mycoplasmas were isolated from conjunctival and/or infraorbital sinus swabs from clinically affected birds. Isolates were identified as Mycoplasma gallisepticum (MG) by direct immunofluorescence and DNA probe-based polymerase chain reactions. These findings suggest that MG is the likely etiology for this epornitic of conjunctivitis in house finches.
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BACKGROUND & AIMS: Empirical therapy has been proposed for initial management of dyspepsia. The aim of this study was to evaluate initial endoscopy, empirical therapy, and testing for Helicobacter pylori in the management of patients with a new onset of dyspepsia. METHODS: Decision analysis was used to compare the direct medical charges in the first year after the onset of dyspepsia for patients managed by initial endoscopy or empirical therapy, with or without initial testing for H. pylori. RESULTS: Medical care charges were $2162.50 for initial endoscopy and $2122.60 for empirical therapy, a difference of 1.8%. For a 55-year-old adult, life expectancy was 23.49 years for initial endoscopy compared with 23.48 years for empirical therapy. Empirical therapy has lower charges than initial endoscopy when H2-receptor antagonists are used to prevent recurrence of dyspepsia. Initial noninvasive testing for H. pylori has lower charges than initial endoscopy if patients with dyspepsia with H. pylori receive antimicrobial therapy without endoscopy but would have higher charges if patients with H. pylori routinely have endoscopy. CONCLUSIONS: Surprisingly, the choice of optimal management strategy was a "toss-up." Only modest savings may result from practice guidelines that recommend empirical therapy in the management of patients with dyspepsia.

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Division of Infectious Diseases, Department of Internal Medicine, Howard University Hospital, 2041 Georgia Avenue NW, Washington, DC 20060, USA.
Stevens-Johnson syndrome (SJS) is an uncommon occurrence in Mycoplasma pneumoniae (M. pneumoniae) infection (1-5%) and has been mainly reported in children and young adults. We present a case of SJS in a 32-year-old male induced by M. pneumoniae infection. This patient presented with fever, cough, and massive occupation of mucus membranes with swelling, erythema, and necrosis accompanied by a generalized cutaneous rash. He clinically responded after treatment with antibiotics and IVIG. SJS is usually a drug-induced condition; however, M. pneumoniae is the commonest infectious cause and should be considered in the differential diagnosis.
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Department of Pediatrics, Triemli Hospital, Zurich, Switzerland Division of Infectious Diseases and Hospital Epidemiology, University Children's Hospital, Zurich, Switzerland Department of Dermatology, Triemli Hospital, Zurich, Switzerland.
Background: Mycoplasma pneumoniae may induce mucosal inflammation, referred to as M. pneumoniae-associated mucositis (MPAM). There is no generally accepted definition of MPAM, since there may be mucosal lesions only, or mucosal and minimal skin lesions. Patients and Methods: We conducted a literature review of MPAM, paying particular attention to pathogenesis, clinical manifestations, treatment decisions, and prognosis. Results: We identified 32 cases of MPAM (median age 13.5 years), whereof 23 patients were otherwise healthy children and young adolescents (72%). M. pneumoniae infection was associated with fever and respiratory symptoms in all calls; it was confirmed by serology (n = 30) and/or PCR (n = 9). Oral lesions were present in all cases, followed by ocular (97%) and uro-genital lesions (78%). Despite the syndrome's name postulating the absence of cutaneous involvement, minimal skin lesions occurred in 31%. Treatment regimens included systemic antibiotics (100%) and systemic anti-inflammatory treatment with corticosteroids (31%) or immunoglobulins (9%). Macrolides were given in 81%, with failure, relapse, and/or worsening in one-third of patients. No patient suffered long-term sequelae. Conclusion: MPAM is a distinct extra-pulmonary manifestation falling into the continuum of Stevens-Johnson syndrome. This entity may be due to inflammatory mechanisms suggesting that systemic anti-inflammatory treatment is even more important than antimicrobials.
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Dermatology Unit, Queen Sirikit National Institute of Child Health, College of Medicine, Rangsit University, Bangkok, Thailand. srisupalak@yahoo.com
BACKGROUND Stevens-Johnson syndrome (SJS) is a rare and severe life-threatening hypersensitivity syndrome. The etiology is unclear but is associated with drug exposure or infections and frequently high morbidity and mortality. OBJECTIVE To determine etiologies, treatments and complications of Stevens-Johnson syndrome (SJS) in children. MATERIAL AND METHOD A retrospective descriptive study was performed at Queen Sirikit National Institute of Child Health during 1979 and 2007 (29-year study). The authors collected and separated data into three phases from 1979 to 1987, 1988 to 1997 and 1998 to 2007. Diagnosis was confirmed by pediatric dermatologists. RESULTS There were 189 patients, 56 cases between 1979-1987, 72 cases between 1988-1997 and 61 cases between 1998-2007. The ratio of male to female was 1.6: 1. The range of age was from 2 months to 15 years old with a mean age of 5.5 years. One hundred and sixty-five cases (87%) had a history of drug taking before onset of the rash. The most common drugs exposure were antibiotics in 69 cases (42%), anticonvulsant drugs in 58 cases (35%), non-steroids anti-inflammatory drugs in 8 cases (5%), antimalarial drugs in 4 cases (2%) and unknown drugs in 26 cases (16%). Mycoplasma infections were found in 5 cases (3%). One hundred and nine cases (58%) were treated with systemic corticosteroids. The corticosteroid treatment was increasing from 18% in the first phase to 64% and 87% in the second and third phase respectively. The overall complications were found in 38 cases (20%) included bacterial skin infections in 16 cases (8%), eye complications in 12 cases (6%), hepatitis in 4 cases (2%) and other complications in 6 cases (2%). Ten patients (5%) died from sepsis and underlying diseases. The mortality rate declined from 9% in the first phase to 1.5% in the third phase. CONCLUSION Etiology of SJS in children was associated with drug exposure with the most commonly implicated drug being antibiotics and anticonvulsants. Corticosteroid may have a role in the treatment of SJS.
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The Children's Hospital at Saint Peter's University Hospital, New Brunswick, NJ, USA.
Papular purpuric gloves and socks syndrome (PPGSS) is a self-limited, often febrile illness with symmetric edema and erythema of the hands and feet; papular, petechial, and purpuric acral dermatosis; and mucosal lesions in children and young adults. Most of the cases of PPGSS have been reported to be caused by parvovirus B19 and other viruses. This study describes a case resulting from Mycoplasma pneumoniae infection in an adolescent male and reviews the literature.
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Department of Pathology, Wake Forest University School of Medicine, Winston-Salem, NC, USA.
Leptomeningeal melanocytosis is a primary melanocytic lesion of the central nervous system that is characterized by diffuse melanocytic infiltration of the leptomeninges. It is seen almost exclusively in children with large congenital nevi and together the findings form a dermatologic syndrome known as neurocutaneous melanosis. We report a rare and atypical case of a 31-year-old adult male with no evident congenital melanocytic lesions who presented with neurologic symptoms and was found to have leptomeningeal melanocytosis. The brain biopsy demonstrated a conspicuous but benign-appearing melanocytic infiltrate that was discordant with the severity of the patient's symptoms. Ultimately, the patient was suspected to represent a case of former fruste neurocutaneous melanosis. Herein the relevant clinical and histopathologic features are discussed along with a brief review of the literature.
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Department of Pediatrics, University of Padova, Padova, Italy.
Mycoplasma pneumoniae-associated mucositis (MPAM), previously labelled as atypical Stevens-Johnson syndrome (SJS), SJS with minimal or no skin manifestations, is a rare non-respiratory manifestation of Mycoplasma pneumoniae infection. The nineteen cases described so far in children and young adults were characterized by a high male gender prevalence (16/19) and a good response to appropriate antibiotic treatment and supportive care in the majority of patients. We describe a case of MPAM in a previously healthy girl, who improved after a 0.5 g/kg daily dose of intravenous immunoglobulins (IVIG) for four consecutive days, after traditional therapy had failed. Conclusion:  The successful treatment with IVIG described in this report suggests that, where appropriate antibiotic and supportive therapy fails to improve the clinical course of severe MPAM, IVIG treatment is worth considering.
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Department of Paediatrics,Triemli Hospital Zurich, Zurich, Switzerland.
Stevens-Johnson syndrome is a severe mucocutaneous disease following drugs or infections. We present a 7-year-old boy with mucous membrane lesions (stomatitis, conjunctivitis, and urethritis) but without skin lesions. The diagnosis of acute Mycoplasma pneumoniae infection strongly suggests a concomitant Fuchs syndrome.
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1Department of Obstetrics and Gynecology, Veterans General Hospital, Kaohsiung; 2Department of Obstetrics and Gynecology, Tzuchi General Hospital, Hualien; 3Department of Pathology, Veterans General Hospital, Kaohsiung; and 4School of Nursing, Fooyin University, Kaohsiung, Taiwan, Republic of China.
Molluscum contagiosum is a common condition that often occurs in children, sexually active young adults, and immunocompromised patients. Generally, molluscum contagiosum lesions are self-limited. They often resolve spontaneously. We report a severe case of genital molluscum contagiosum in a patient with Sjögren syndrome. Her vulvar lesions responded to topical 5% imiquimod cream.
Cases J. 2009 ;2 :7748  19918477 
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[My paper] Matthew J Booker
School of Education, University of Birmingham Edgbaston, Birmingham, B15 2TT UK. mjb072@bham.ac.uk
This case report describes a 41-year-old Afro-Caribbean lady presenting with a constellation of pyrexia, conjunctivitis, arthralgia, sterile dysuria, apthous ulceration, labial crusting and widespread erythema multiforme. A diagnosis of Stevens-Johnson Syndrome was made. She had taken no medications recently (the most common precipitant of Stevens-Johnson Syndrome) and a full screen for the common and atypical bacterial and viral triggers was negative. The identified trigger was the use of a chemical hair relaxant treatment a few days previously. With supportive measures and a course of oral prednisolone, the patient quickly improved and made a full recovery. This case highlights the importance of considering occupational and recreational precipitants of Stevens-Johnson Syndrome.
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Unidad de Infecciosos, Servicio de Pediatría, Hospital Clínico Félix Bulnes, Santiago, Chile. julia.villarroel@vtr.net
The mucocutaneous manifestations of Mycoplasma pneumoniae infection appear in approximately 20% of all the infections produced by this microorganism. Maculopapular rash, vesicular or urticarial exanthemas, are frequent manifestations that can constitute Erythema multiforme or more rarely, Stevens-Johnson syndrome or epidermal toxic necrolisis. We describe the clinical evolution, diagnosis and treatment of four children with mucous and cutaneous manifestations associated to infection by Mycoplasma pneumoniae and a review of the medical literature.


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