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.

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.