Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use. Sections Introduction Methods Recommendations Conclusions

Our cohort derived considerable benefit from rituximab permitting a reduction in immunosuppressive exposure and prednisolone dose with few major adverse effects. There was an 80% (4/5) response in patients with retro-orbital granulomas. The effect of rituximab was most noticeable in the first 6 months (88% response).

We would recommend a full review of all histological specimens in patients with a diagnosis of temporal bone inflammatory pseudotumour or inflammatory myofibroblastic tumour. Consideration should be given to immunohistochemical analysis for anaplastic lymphoma kinase and immunoglobulin G4, with measurement of serum levels of the latter. Management of the condition is medical, with corticosteroids and immunosuppression, rather than surgical excision.

Malakoplakia is a rare inflammatory condition that typically occurs in the urinary tract. The cutaneous form is less prevalent, and most commonly occurs in the perianal or genital regions. Here we present a 61 year old lady with cutaneous malakoplakia of the neck, which was successfully treated with surgical excision and a prolonged course of ciprofloxacin. We follow our case report with a discussion and literature review of all seventeen previously reported cutaneous head and neck malakoplakia cases from the literature. A diagnosis of cutaneous malakoplakia should be considered in nodular, ulcerated or discharging lesions that are refractory to treatment. Histology is essential, not only to diagnose malakoplakia, but also to exclude other important differential diagnoses, such as malignancy. Combined surgical excision and prolonged antibiotic courses appear to have the highest success rate. Antibiotics should be culture specific, but quinolones appear to be the best empirical choice.