Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms severity score: A useful tool for assessing disease severity and predicting fatal cytomegalovirus disease.

BACKGROUND: The prognosis of drug-induced hypersensitivity syndrome (DiHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) is highly unpredictable. Severe complications, either related or unrelated to cytomegalovirus (CMV) reactivation, are a highly probable cause of death. OBJECTIVES: The aim was to establish a scoring system for DiHS/DRESS that can be used to monitor severity, predict prognosis, and stratify the risk of developing CMV disease and complications. METHODS: A retrospective analysis of 55 patients with DiHS/DRESS was performed. A composite score was created using clinical data. DiHS/DRESS patients were also stratified into 3 groups based on the scores to predict the risk of CMV reactivation and complications. RESULTS: This scoring system made it possible to predict CMV disease and complications. Scores ≥4 were associated with the later development of CMV disease and complications, while no patients with scores <4 developed complications. LIMITATIONS: This was a single-institution study with a relatively small patient cohort that lacked a validation cohort. CONCLUSIONS: Our scoring system may be useful for predicting CMV-related complications, and early intervention with anti-CMV agents should be considered in patients with scores ≥4 or with evidence of CMV reactivation.

Bullous pemphigoid complicated by cytomegalovirus disease as a manifestation of immune reconstitution inflammatory syndrome: retrospective analyses of our institutional cases and literature review.

BACKGROUND: Cytomegalovirus (CMV) disease induced by reactivation of latent CMV is a fatal viral infection that may develop in a setting of therapy with immunosuppressive agents. There is a clear need to clarify any clinical features and markers of CMV disease. OBJECTIVE: We investigated which clinical markers usually available in a clinical setting can predict CMV disease occurring in bullous pemphigoid (BP) patients receiving corticosteroids. METHOD: We described a BP patient with CMV disease complicated by gastrointestinal hemorrhage and liver dysfunction. Prompted by this patient, we retrospectively analyzed clinical features and laboratory findings in our institutional four BP patients and previously reported nine BP patients with CMV disease. We also compared these patients with our institutional 42 BP patients not complicated by CMV disease. RESULTS: High levels of anti-BP180 antibody titers associated with resistance to corticosteroids are a risk factor for the development of CMV disease. A reduction in platelet (PLT) and white blood cell (WBC) counts and an increase in alanine aminotransferase (ALT) levels 3-4 weeks after the initiation of corticosteroids are useful predictive markers for the onset of CMV disease. CONCLUSIONS: Frequent WBC, PLT, and ALT measurements may identify BP patients at a risk of subsequently developing CMV disease. Careful monitoring of CMV disease in BP refractory to systemic corticosteroids may reduce the risk of fatal outcomes.

Differences in clinical features and outcomes between group A and group G Streptococcus -induced cellulitis.

BACKGROUND: Streptococci are the main causative agents of cellulitis, and group G Streptococcus (GGS) shares many important virulent factors with group A Streptococcus (GAS). The difference in the clinical features of GAS- and GGS-induced cellulitis, however, has not been thoroughly characterized. OBJECTIVE: Our aim was to recognize the differences in the clinical manifestations and outcomes of lower limb cellulitis caused by GAS and GGS. METHODS: We retrospectively analyzed a total of 29 patients diagnosed with GAS- or GGS-induced lower limb cellulitis during the period from January 2008 to September 2013. RESULTS: While the clinical manifestations of GAS-induced cellulitis were likely to be uniform, those of GGS-induced cellulitis were variable, depending on the predisposing factors. GGS-induced cellulitis occurred more frequently in older person who had chronic underlying illness. CONCLUSION: We identified clinical predisposing factors that can predict the clinical course and outcomes of GGS-induced cellulitis.

Short- and long-term outcomes of 34 patients with drug-induced hypersensitivity syndrome in a single institution.

BACKGROUND: Drug-induced hypersensitivity syndrome (DIHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe systemic hypersensitivity reaction caused by specific drugs, in which herpesvirus reactivations and organ dysfunction occur during the course of the disease. Although recent reports have documented the development of autoimmune disease after complete resolution of DIHS/DRESS, relatively little is known about long-term outcomes after complete resolution of the disease. OBJECTIVE: The aim of this study was to retrospectively analyze complications and sequelae in the early and late phases of DIHS/DRESS according to treatment. METHODS: In all, 34 patients were classified into 2 groups: 14 patients with oral corticosteroid treatment; and 20 with noncorticosteroid treatment. The disease time course was divided into 2 periods: the first 6 months after onset of the drug reaction (early phase); and the period thereafter (late phase). Investigations to detect the presence of viral/bacterial infectious diseases, organ dysfunction, and autoantibodies were performed in both early and late phases. RESULTS: Herpesvirus infections and pneumonia were detected in 6 and 2 patients, respectively, in the corticosteroid treatment group in the early phase. In the noncorticosteroid treatment group, 2 patients developed autoimmune diseases, namely lupus erythematosus and autoimmune thyroiditis. Autoantibodies were detected in 44.4% of patients examined in the late phase of the disease. LIMITATIONS: This study only evaluated a small number of autoantibodies. CONCLUSION: The need for anti-inflammatory effects from systemic corticosteroids should be balanced with the risk of infectious diseases and the benefits of preventing the appearance of later autoimmune conditions in patients with DIHS/DRESS.

Relationship between cytomegalovirus reactivation and dermatomyositis.

Dermatomyositis (DM) is an autoimmune disease manifested by muscle weakness and characteristic cutaneous eruptions. Cytomegalovirus (CMV) belongs to the ß-herpesvirinae subfamily of herpesviridae that cause morbidity and mortality in immunocompromised patients. With respect to the relationship between CMV and DM, it remains unknown whether CMV plays a pathogenetic role or whether CMV disease is an opportunistic infection due to immunosuppressive treatment. We report two patients with DM who developed cutaneous CMV ulcers within one month after the initiation of systemic corticosteroid treatment. In this context, we retrospectively studied the clinical characteristics of six DM patients with CMV reactivation and the effect of corticosteroid treatment on CMV reactivation in these patients. We also examined possible predictive parameters of CMV reactivation during the course of DM. Our results suggest that CMV reactivation occurs more frequently in DM patients than previously recognized; CMV reactivation occurs regardless of the dosage and duration of corticosteroid administration or the presence of underlying disease. Furthermore, our study shows that a reduction in platelets, serum globulin and IgG levels during the course of DM may be useful predictive parameters for CMV reactivation in patients with DM.

Efficacy of plasmapheresis for the treatment of severe toxic epidermal necrolysis: Is cytokine expression analysis useful in predicting its therapeutic efficacy?

Toxic epidermal necrolysis (TEN) is a life-threatening, drug-induced disorder characterized by severe epidermal injury. Although there is no standard therapeutic intervention in TEN, plasmapheresis (PP) is being used increasingly to treat extremely ill TEN patients. In addition to conventional PP, double-filtration PP (DFPP) has been recently used for severe and refractory TEN. In this review, we focus on the clinical usefulness of PP by both demonstrating three cases of TEN refractory to conventional therapies, who were successfully treated with conventional PP or DFPP, and evaluating its therapeutic efficiency. We also provide evidence to suggest the mechanisms of action of PP by investigating the correlation between disease intensity and serum cytokine levels before and after treatment with PP or DFPP in these patients with TEN. At present, PP is a much more effective option for treatment of severe and/or recalcitrant TEN than any other treatment, such as pulsed corticosteroids and i.v. immunoglobulin.

Visceral involvements and long-term sequelae in drug-induced hypersensitivity syndrome.

Drug-induced hypersensitivity syndrome (DIHS) is a severe systemic reaction with several herpesvirus reactivations. Multiple organ failures appear during the course of the disease. The severity of DIHS is determined by the degree of visceral involvement. Autoimmune diseases also develop several months to years after the apparent clinical resolution of DIHS.

Mycoplasma pneumoniae infection-induced erythema nodosum, anaphylactoid purpura, and acute urticaria in 3 people in a single family.

Mycoplasma pneumoniae infection is one of the most common etiologic agents of respiratory tract diseases. Although the respiratory symptoms of this infection commonly are mild, it often is accompanied by various extrapulmonary complications including arthritis and cutaneous manifestations. We report 3 patients with M pneumoniae infection in a single family who revealed erythema nodosum, anaphylactoid purpura, and acute urticaria, respectively. We discuss the similarity between these cutaneous manifestations caused by this infection and those caused by viral infections, and the responsible factors for producing different cutaneous lesions by a single infectious agent in people with common genetic background. Age-related variations in cutaneous manifestations of M pneumoniae infections can be attributed to the immaturity of the adaptive immunity of a host, as has been suggested in viral infections.