Mortality and risk factors on admission in toxic epidermal necrolysis: A cohort study of 59 patients.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening disorders characterized by widespread epidermal necrosis of the skin and mucosa. The severity-of-illness scoring system for TEN (SCORTEN) was widely used since 2000 as a standard prognostic tool consisting of seven clinical values. METHODS: To evaluate the prognosis using current treatments and risk factors for mortality, we retrospectively analyzed 59 cases of TEN, including SJS/TEN overlap treated in two university hospitals from January 2000 to March 2020. RESULTS: The mortality rate of TEN was 13.6% (8/59). All patients treated with high-dose steroid administration in combination with plasma exchange and/or immunoglobulin therapy recovered. Logistic regression analysis showed nine clinical composite scores, namely: heart rate (≧120 bpm), malignancy present, percentage of body surface area with epidermal detachment (>10%), blood urea nitrogen (>28 mg/dL), serum bicarbonate level (<20 mEq/L), serum glucose level (>252 mg/dL), age (≧71 years), the interval between disease onset and treatment initiation at the specialty hospital (≧8 days), and respiratory disorder within 48 h after admission. The receiver operating characteristic curves confirmed a high potential for predicting the prognosis of TEN. CONCLUSIONS: Recent developments in treatment strategies have contributed to the improved prognosis of TEN patients. A modified severity scoring model composed of nine scores may be helpful in the prediction of TEN prognosis in recent patients. Further large-scale studies are needed to confirm mortality findings to improve prognostication in patients with TEN.

Retrospective analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis in 87 Japanese patients--Treatment and outcome.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe adverse drug reactions with high mortality. METHODS: To present the clinical characteristics of SJS and TEN in Japan and evaluate the efficacy of treatments, we retrospectively analyzed cases of SJS and TEN treated in 2 university hospitals during 2000-2013. RESULTS: Fifty-two cases of SJS (21 males and 31 females; average age, 55.1 years) and 35 cases of TEN (17 males and 18 females; average age, 56.6 years) were included in this study. Twenty-eight cases of SJS (53.8%) and all cases of TEN were caused by drugs. Hepatitis was the most common organ involvement in both SJS and TEN. Renal dysfunction, intestinal disorder, and respiratory disorder were also involved in some cases. The major complication was pneumonia and sepsis. All cases except for 3 cases were treated systemically with corticosteroids. Steroid pulse therapy was performed in 88.6% of TEN. Plasmapheresis and/or immunoglobulin therapy was combined with steroid therapy mainly in TEN after 2007. The mortality rate was 6.9% and the rates for SJS and TEN were 1.9% and 14.3%, respectively. These were much lower than predicted mortality according to a severity-of-illness scoring system for TEN prognosis (SCORTEN) score. When comparing the mortality rate between 2000-2006 and 2007-2013, it was decreased from 4.5% to 0.0% in SJS and from 22.2% to 5.3% in TEN. CONCLUSIONS: Treatment with steroid pulse therapy in combination with plasmapheresis and/or immunoglobulin therapy seems to have contributed to prognostic improvement in SJS/TEN.

Myocardial scintigraphy may predict the conversion to probable dementia with Lewy bodies.

OBJECTIVE: To compare the usefulness of brain perfusion SPECT and (123)I-metaiodobenzylguanidine ((123)I-MIBG) in predicting the conversion of possible dementia with Lewy bodies (DLB) to probable DLB. METHODS: We examined 94 patients with possible DLB based on the Consensus Criteria for the Clinical Diagnosis of DLB by N-Isopropyl-p-(123)I-iodoamphetamine ((123)I-IMP) brain perfusion SPECT and (123)I-MIBG myocardial scintigraphy. After 1 year of follow-up, 33 of 94 patients met the criteria for probable DLB. (123)I-IMP brain perfusion SPECT and (123)I-MIBG myocardial scintigraphy were tested as predictors of the conversion from possible DLB to probable DLB. A receiver operating characteristic (ROC) analysis was performed. RESULTS: The areas under the ROC curves for SPECT for predicting the conversion to probable DLB from possible DLB based on the occipital/cerebellum and occipital/striatum cortex ratios of blood flow counts were 0.591 and 0.585, respectively. The areas under the ROC curves for (123)I-MIBG based on the early heart to mediastinum (H/M) ratio, delayed H/M ratio, and washout rate were 0.935, 0.936, and 0.884, respectively. CONCLUSION: (123)I-MIBG myocardial scintigraphy is a good predictor of the future conversion of possible DLB to probable DLB.

[Depression in dementia with Lewy bodies].

Depression is a risk factor for dementia in general, including Alzheimer's disease (AD), its premorbid signs are commonly observed, and the morbidity of depression is higher in dementia patients. Dementia with Lewy bodies (DLB) is considered to have an even higher depression prevalence and premorbid depression rate than other dementias such as AD. This led to depression being listed as a supportive feature in the 2005 criteria for the clinical diagnosis of DLB. However, studies investigating the difference in depression between AD and DLB failed to show consistent results. We examined the Geriatric Depression Scale score, which is designed specifically to rate depression in the elderly, for DLB and AD patients. The scores for DLB patients were twice as high as those for AD patients. There was no correlation between the GDS score and age, sex, or Mini-Mental Sate Examination scores. Depression-specific symptoms were more frequent in the DLB group than non-specific symptoms, while less than one third of DLB patients with very high GDS scores were diagnosed with depression or prescribed antidepressants for depressive symptoms. Other researchers reported that depression of DLB was associated with a higher prevalence of psychiatric symptoms other than major depression, and suggested that depression of DLB might be a part of psychiatric syndrome. There has been no systematic study on the validity or risk of pharmacological therapy, as well as the necessity of intervention, for depression or a high GDS score in DLB. Therefore, intervention must rely on the clinical decision of each doctor. In spite of the paucity of current findings, studies on depression of DLB may play a key role in the elucidation of its neuropathology and psychopathology and offer a new view point on understanding depression itself.

Statistical analysis of Stevens-Johnson syndrome caused by Mycoplasma pneumonia infection in Japan.

BACKGROUND: Stevens-Johnson syndrome (SJS) associated with Mycoplasma pneumoniae (M. pneumoniae) infection is mainly observed in children. In adults, drugs are a major cause of SJS, but some adult patients with SJS are infected with M. pneumoniae. We analyzed patients with SJS associated with M. pneumoniae infection to elucidate the differences between drug-induced SJS and M. pneumoniae-associated SJS and also to study differences between M. pneumoniae-associated SJS in children and adults. METHODS: This is a retrospective review of Japanese patients who have been reported as M. pneumoniae-associated SJS in medical Journals published from 1981 to 2009, compared with data of Japanese patients with drug-induced SJS reported from 2000 to 2009. RESULTS: Thirty-eight cases of M. pneumoniae-associated SJS and 78 cases of drug-induced SJS were analyzed in this study. Ocular lesions were observed more frequently in M. pneumoniae-associated SJS than in drug-induced SJS (p < 0.01), and adult patients showed a higher ratio of sequelae in their eyes than did patients under 20 years of age (p < 0.01). Sixty-six percent of adult patients with M. pneumoniae-associated SJS developed fever/respiratory symptoms and mucocutaneous lesions on the same day. In contrast, most of the patients under 20 years of age developed fever/respiratory symptoms before mucocutaneous involvement. This means that these adult patients were infected and immunized previously and developed allergic reactions to M. pneumoniae soon after the later infection. CONCLUSIONS: In order to prevent ocular sequelae in adult patients when M. pneumoniae infection is suspected, more intensive treatment may be needed in adult patients than in younger patients.

Dementia with Lewy bodies is associated with higher scores on the Geriatric Depression Scale than is Alzheimer's disease.

BACKGROUND: Several reports suggest a higher morbidity of depression in patients with dementia with Lewy bodies (DLB) than in patients with Alzheimer's disease (AD). However, these results have not been duplicated consistently. The psychiatric symptoms of dementia, including depression, are important for its diagnosis and management. Thus, the aim of the present study was to clarify the characteristics of the depressive symptoms in DLB compared with AD using the Geriatric Depression Scale (GDS). METHODS: We examined the GDS score for 86 patients with probable DLB (based on the Consensus Criteria for the clinical diagnosis of DLB) and 86 patients with probable AD (based on criteria of the National Institute for Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association), who were matched according to age, sex, education, and Mini-Mental State Examination (MMSE) scores. We also examined correlations between GDS scores and age, sex, or MMSE scores in both groups. Correlations between GDS scores and metaiodobenzylguanidine (MIBG) scintigraphy were examined in patients with DLB. To characterize the GDS in DLB, its profile was examined using factor structures. RESULTS: Scores for DLB patients were twice as high on the GDS as those for AD patients. There was no correlation between GDS score and age, sex, or MMSE scores in either group. Furthermore, there was no correlation between the results of MIBG scintigraphy and GDS scores in the DLB group. Using factor structures, the depression symptom profile of these diseases suggested that depression-specific symptoms, such as mood, worry, or future outlook, were more frequent in the DLB group than non-specific symptoms, such as lack of energy, decreased concentration, or apathy. CONCLUSIONS: The data suggest that depressive symptoms are highly specific symptoms of DLB, independent of other features of this disorder. The GDS could be used as a subsidiary tool in differentiating DLB from AD and is more useful than clinical observations of depression.

[Experience of using a filmless system in dental practice at Hiroshima University Hospital, a former university dental hospital].

Our university hospital has had a policy of electronic patient recordkeeping, replacing paper-based recordkeeping, since September 1, 2008. Because the Department of Oral and Maxillofacial Radiology had already been using computed radiography for X-ray systems except for intraoral radiography and storing data in Digital Imaging and Communications in Medicine (DICOM) format, the following three conditions form the basis of the changes we made in relation to the introduction of this policy. We started 1. using imaging plates for intraoral radiography as well and storing the data in DICOM format; 2. diagnosed without the need for film; and 3. referred to past images displayed on the screen of our Radiology Information System (RIS). The introduction of digital intraoral radiography has many advantages: not only does it not require film and can all past images be referred to on the screen of the RIS, but radiation exposure times are also shorter, images can be saved electronically, and thus, film processing is redundant. The system improves efficiency and is also advantageous to patients and staff in other departments.

[Analysis of treatments and deceased cases of severe adverse drug reactions--analysis of 46 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis].

BACKGROUND: Systemic administration of corticosteroid, plasmapheresis and high-dose immunoglobulin therapy (IVIG) are the main treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). OBJECTIVE: To evaluate the effects of the treatments of SJS and TEN. METHODS: Twenty-seven cases of SJS and 19 cases of TEN treated in our hospitals from 2000 to 2007 were analyzed. RESULTS: Corticosteroid was administered systemically in all cases except one case of TEN which developed methicillin resistant staphylococcus aureus (MRSA) pneumoniae before the onset of the eruption. Methylprednisolone (mPSL) pulse therapy (1000 mg/day) or mini pulse therapy (less than 600 mg/day of mPSL) was selected by 8 cases of SJS and 9 cases of TEN. Combination of plasmapheresis or IVIG with corticosteroid therapy was performed in 3 cases of SJS and 8 cases of TEN. The mortality rate of patient with SJS was 3.7% (1 case), and with TEN was 21.1% (4 cases). The deceased case of SJS had been treated with corticosteroid alone and died for acute respiratory disorder after 24 days from the onset of the eruption. Four deceased cases of TEN were treated with corticosteroids with or without IVIG, and 2 of them merged sepsis. CONCLUSIONS: Although corticosteroids may enhance the risk of sepsis, prompt treatment with systemic corticosteroids seems to reduces morbidity and improves outcome of SJS and TEN patients.

New horny layer marker proteins for evaluating skin condition in atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) has a complicated pathogenesis and its clinical features vary greatly among patients. Although many clinical parameters have been reported, it remains difficult to evaluate AD skin conditions adequately. OBJECTIVE: To support better evaluation of AD patients, we attempted to develop a new, objective and noninvasive method that assesses skin condition in AD using biochemical markers in the skin's horny layer (HL). METHODS: Thirty-six patients with AD, 8 with psoriasis and 16 healthy volunteers were recruited. HL samples were obtained by tape stripping from involved and uninvolved skin of the forearms. Expression levels of 6 proteins in the HL [fatty acid-binding protein-5 (FABP-5), squamous cell carcinoma antigens 2 (SCCA2), alpha-enolase, annexin II, apolipoprotein A-I and albumin] were analyzed by immunoblotting and compared with clinical data. RESULTS: The 6 proteins were detected at a high level in AD skin lesions, but scarcely in the normal controls. FABP-5 showed correlation with the local severity of the involved skin. Annexin II, apoprotein A-I and albumin showed correlation with the severity of specific eruptions. SCCA2 correlated significantly with total serum IgE level. Albumin levels in the uninvolved skin of AD patients showed significant correlation with the local severity in the involved skin of the same patient and with the trans-epidermal water loss. Albumin levels in psoriatic skin were very low, even with scratch marks, compared to those in AD skin. CONCLUSION: FABP-5, albumin and some other proteins in HL seem to be useful as biomarkers to evaluate inflammation and skin barrier conditions in AD patients.

Global cerebral hypoperfusion in a patient with transient global amnesia.

We report a 64-year-old male patient with pure transient global amnesia (TGA) who exhibited global cerebral hypoperfusion during an amnesia attack. Initial single photon emission computed tomography (SPECT) performed 3 hours after the onset of the amnesic attack revealed diffuse hypoperfusion in the cerebrum, whereas a second SPECT study, 1 month later, revealed improvement of cerebral blood flow. This case suggests that SPECT study is useful in revealing the pathophysiological mechanism of TGA and that TGA attacks are associated with global cerebral blood flow change.

[Oral allergy syndrome due to plant-derived foods: a clinical review of 63 patients over a period of 6 years].

BACKGROUND: The clinical features of many patients with oral allergy syndrome (OAS) due to plant-derived foods have rarely been reported in Japan. OBJECTIVES: We aimed to determine the causative foods of OAS due to plant-derived foods based on clinical features and skin prick tests (SPTs). Furthermore, we aimed to elucidate the association between causative foods and sensitized pollens in patients with OAS due to plant-derived foods. METHODS: SPTs and specific IgE measurements (CAP-FEIA: CAP) were performed in relation to foods and pollens in 118 patients with positive histories of OAS due to plant-derived foods. Patients with positive histories and with positive skin test responses were identified as having type I allergy to the causative foods. RESULTS: The mean age of 63 patients with positive histories and positive skin test responses was 29.2 years (range, 2-61 years), and there were twice as many females as male. The most frequent causative foods were found to be apple, peach, kiwi, and melon in 13, 12, 12, and 11 patients, respectively. CAP frequency was shown to be similar to that of SPT regarding apple, whereas it was less than that of SPT regarding melon, peach, and kiwi. A significant correlation between the frequencies of SPT and CAP was found regarding apple (r=0.39, p<0.05) but not peach, kiwi, and melon. Forty-one of 63 patients with OAS (66.1%) had pollinosis and/or allergic rhinitis. In patients with OAS due to apple, the positive ratio of CAP response against alder pollen was higher than that in patients with OAS due to melon. In patients with OAS due to melon, the positive ratio of CAP responses against ragweed pollen, grass pollen, and mugwort pollen was higher than that in patients with OAS due to apple. CONCLUSION: In this study, positive ratios of SPT and CAP tended to differ according to the causative food, showing a smaller potential for reaction than might be suggested by patient history. Therefore, for the time being it would be more accurate to use a skin test for the diagnosis of OAS due to plant-derived foods.

Analysis of Stevens-Johnson syndrome and toxic epidermal necrolysis in Japan from 2000 to 2006.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse drug reactions with high mortality. METHODS: To present the current clinical characteristics and treatment of SJS and TEN in Japan, we retrospectively analyzed reports of SJS and TEN published in medical journals from 2000 to 2006. RESULTS: Fifty-two cases of SJS (19 males and 33 females; mean age, 45.2 years) and 65 cases of TEN (31 males and 34 females; mean age, 45.7 years) were reported. Thirty-six cases of SJS (69.2%) and all cases of TEN were caused by drugs, such as nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics, and anticonvulsant drugs. Hepatitis was the most common organ involvement in both SJS and TEN. Renal dysfunction and respiratory disorders were also involved in some cases. The major complication was sepsis, but in only 1.9% of SJS and 10.8% of TEN. Most cases were treated systemically with corticosteroids, and 42 cases (80.8%) of SJS and 39 cases (60.0%) of TEN were treated with corticosteroids alone. Plasmapheresis and/or immunoglobulin therapy was combined with corticosteroid therapy in some cases. The mortality rates for patients with SJS and TEN were 1.9% and 6.2%, respectively. The mortality in TEN decreased remarkably from 21.6% (58/269) during the previous 17 years (1981 to 1997). CONCLUSIONS: Improvement of treatment may be one of the reasons for the decrease in mortalities of both SJS and TEN.

[Enhancement of nonsteroidal, anti-inflammatory drugs and preventive effect of antihistamines and disodium cromoglycate on wheat allergy].

BACKGROUND: Aspirin has been known to be an enhancer to wheat allergy, including wheat-dependent, exercise-induced anaphylaxis. OBJECTIVE: To investigate whether nonsteroidal, anti-inflammatory drugs (NSAIDs) other than aspirin would enhance allergic reactions after wheat ingestion and whether antihistamines and disodium cromoglycate would prevent these reactions. METHODS: Seven cases, whose reactions after wheat ingestion were enhanced by aspirin on challenge tests, were enrolled. Skin prick tests (SPT) and CAP-RAST were undergone for wheat and gluten. We used challenge tests of wheat after pretreatment of NSAIDs and preventive drugs. RESULTS: Four cases were diagnosed with wheat allergy, 3 cases had wheat-dependent, salicylic acid-induced anaphylaxis. SPT and CAP-RAST were positive for wheat and gluten in 5 of 7 cases and 4 of 7 cases, respectively. Dicrofenac enhanced the allergic reactions after wheat ingestion in 1 of 2 cases, whereas etodolac failed to enhance the symptoms in all 5 cases performed. Furthermore, disodium cromoglycate could not completely prevent the allergic reaction in all 4 cases and even enhanced the reaction in 1 case of them. To see an inhibitory effect of antihistamines on the symptoms, fexofenadine (in 2, 1 and 1 case, respectively), olopatadine, and chlorpheniramine were administrated before the challenge test, and as a result these drugs were found to have inhibitory effects on the allergic reaction. CONCLUSION: In this study, it was suggested that etodolac might be a relatively safe anti-inflammatory drug on wheat allergy and antihistamines could prevent allergic reactions more than DSCG in patients with wheat allergy.