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BackgroundEcthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18 expression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal infections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection.Case PresentationWe report at our knowledge the first case of a child affected by LAD-1, who experienced during her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted antibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes pockets transfusions in association with G-CSF had the capacity to cure this lesion.ConclusionWe propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.

Allergy to insulin became a rare complication due to the introduction of recombinant human insulin preparations. Nevertheless, allergic reactions to components of such preparations can occur. We report a case of a 61-year-old man with an atopic background and affected by diabetes mellitus type 2 since 27 years, who experienced generalized allergy to insulin at the moment of switching oral anti-diabetics to insulin. Prick tests revealed an allergy specifically to zinc, and the patient was treated with zinc-free glulisine insulin. After 8 months of such treatment, patient's glucose is stable and he never experienced allergic reactions to insulin injections. Even insulin allergy due specifically to zinc is rare, such complication must be assessed especially in a patient suffering from multiple allergies.

Introduction: Serum immunofixation electrophoresis (sIFE) and serum free light chains (sFLC) immunoassays are fundamental for serum monoclonal protein diagnosis and monitoring. In this study, we compared the ability of these assays to detect monoclonal proteins in stem cell transplanted patients. Methods: Three hundred sixty sera belonging to 145 patients affected by MM after autologous stem cell transplantation [113 intact immunoglobulin multiple myeloma (IIMM) and 32 free light chains multiple myeloma (LCMM)] were analyzed by both sIFE and sFLC assay, respectively performed with the Binding Site Minifix Kit and the Binding Site Freelite kit on Cobas-Integra 400 Plus (Roche).Results: Two hundred one samples (55.8%) had an abnormal κ/λ ratio and 32.7% had a monoclonal restricted band in sIFE. For 61.38% samples, there was a concordance between sIFE and sFLC and there wasn't for 139 samples (p<0.0001).Kappa Cohen's coefficient was weak (κ= 0.25). When compared to sFLC immunoassay for IIMM, sIFE demonstrated a weak sensitivity of 80.7%, which increases to 94.74% when we compared only LCMM results. Accuracy of sIFE respect to sFLC was the better for IIMM (70.7%), than for all type MM (61.4%), and there was a significant difference between the accuracy of either of these techniques, when performed singly for all MM type, IIMM and LCMM.Conclusion: sFLC assay role in IIMM monitoring is to be confirmed by further studies. sFLC assay is a sensitive tool for the monitoring of patients after transplantation, and that it may be associated to sIFE assay to better individualizing minimal residual disease.