Pyoderma gangrenosum (PG) is an uncommon, chronic, painful, ulcerative skin disease of unknown origin, often associated with systemic diseases including inflammatory bowel disease, rheumatoid arthritis; monoclonal gammopathy, hepatitis and myeloproliferative disorders. The mainstay of therapy for PG has been high-dose corticosteroids but not all patients have a favourable outcome. Other systemic agents have also been used such as cyclosporine, azathioprine, cyclophosphamide and tacrolimus. However, all these systemic therapies can be complicated by serious side effects. In this paper, we report a series of four patients with pyoderma gangrenosum treated successfully with topical cyclosporine. No side-effects were observed in any of the patients and there was minimal systemic absorption of the topical cyclosporine.
Autoimmune hepatitis is an inflammatory condition of the liver that can lead to significant morbidity and mortality. Corticosteroids with or without azathioprine have been shown to improve outcome and are the current standard of care in autoimmune hepatitis patients. However, long-term use of corticosteroids and use of azathioprine could be associated with significant adverse effects that prevent their continued use at optimal dosages or may even require complete cessation. We present a patient with autoimmune liver cirrhosis who was intolerant of corticosteroid and azathioprine, who was successfully treated with cyclosporine. To our knowledge, cyclosporine use has not been reported previously in autoimmune cirrhosis, although it has been used in autoimmune hepatitis patients with reported success and good tolerability. We conclude that cyclosporine seems to be an effective alternative to azathioprine as a steroid-sparing agent in both non-cirrhotic and cirrhotic autoimmune hepatitis.
Hemophagocytic lymphohistiocytosis (HLH) is a disorder characterized by uncontrolled mature histiocyte proliferation, hemophagocytosis, and hypercytokinemia. We describe a previously healthy pregnant patient who presented in the third trimester of pregnancy with HLH.
The influence of optical parameters, additional techniques (e.g. PIDS technology) and the importance of light microscopy were investigated by comparing laser diffraction data obtained via the conventional method and an optimized analysis method. Also the influence of a possible dissolution of nanocrystals during a measurement on the size result obtained was assessed in this study. The results reveal that dissolution occurs if unsaturated medium or microparticle saturated medium is used for the measurements. The dissolution is erratic and the results are not reproducible. Dissolution can be overcome by saturating the measuring medium prior to the measurement. If nanocrystals are analysed the dispersion medium should be saturated with the nanocrystals, because the solubility is higher than for coarse micro-sized drug material. The importance of using the optimized analysis method was proven by analysing 40 different nanosuspensions via the conventional versus the optimized sizing method. There was no large difference in the results obtained for the 40 nanosuspensions using the conventional method. This would have led to the conclusion, that all the 40 formulations investigated are physically stable. However, the analysis via the optimized method revealed that from 40 formulations investigated only four were physically stable. In conclusion an optimized analysis saves time and money and avoids misleading developments, because discrimination between "stable" and "unstable" can be done reliably at a very early stage of the development.