BackgroundLeprosy Type 1 (T1R) reactions are immune-mediated events leading to nerve damage and preventable disability affecting hands, feet and eyes. Type 1 Reactions are treated with oral corticosteroids. There is little evidence on alternative treatments for patients who do not respond to steroids or experience steroid adverse effects. We report the results of a randomized controlled trial testing the efficacy and adverse effect profile of ciclosporin and prednisolone (CnP) in comparison to prednisolone only (P) in patients with new T1R in Ethiopia. Ciclosporin is a potent immunosuppressant. Outcomes were measured using a clinical severity score, recurrence rate, adverse events and quality of life.ResultsSeventy three patients with new T1R were randomized to receive CnP or P for 20 weeks. Recovery rates in skin signs was similar in both groups (91% vs 88%). Improvements in nerve function both, new and old, sensory (66% vs 49%) and motor (75% vs 74%) loss were higher (but not significantly so) in the patients on CnP. Recurrences rates of T1R (85%) were high in both groups, and recurrences occurred significantly earlier (8 weeks) in patients CnP, who needed 10% more additional prednisolone. Serious major and minor adverse events rates were similar in patients in the two treatment arms of the study. Both groups had a significant improvement in their quality of life after the study, measured by the SF-36.ConclusionsThis is the first double-blind RCT assessing ciclosporin, in the management of T1R in Africa. Ciclosporin could be a safe alternative second-line drug for patients with T1R who are not improving with prednisolone or are experiencing adverse events related to prednisolone. This study illustrates the difficulty in switching off leprosy inflammation. Better treatment agents for leprosy patients with reactions and nerve damage are needed.

ObjectivesWe wished to validate our recently devised 16-item ENLIST ENL Severity Scale, a clinical tool for measuring the severity of the serious leprosy associated complication of erythema nodosum leprosum (ENL). We also wished to assess the responsiveness of the ENLIST ENL Severity Scale in detecting clinical change in patients with ENL.MethodsParticipants, recruited from seven centres in six leprosy endemic countries, were assessed using the ENLIST ENL Severity Scale by two researchers, one of whom categorised the severity of ENL. At a subsequent visit a further assessment using the scale was made and both participant and physician rated the change in ENL using the subjective categories of “Much better”, “somewhat better”, “somewhat worse” and “much worse” compared with “No change” or “about the same”.Results447 participants were assessed with the ENLIST ENL Severity Scale. The Cronbach alpha of the scale and each item was calculated to determine the internal consistency of the scale. The ENLIST ENL Severity Scale had good internal consistency and this improved following removal of six items to give a Cronbach’s alpha of 0.77. The cut off between mild ENL and more severe disease was 9 determined using ROC curves. The minimal important difference of the scale was determined to be 5 using both participant and physician ratings of change.ConclusionsThe 10-item ENLIST ENL Severity Scale is the first valid, reliable and responsive measure of ENL severity and improves our ability to assess and compare patients and their treatments in this severe and difficult to manage complication of leprosy.The ENLIST ENL Severity Scale will assist physicians in the monitoring and treatment of patients with ENL. The ENLIST ENL Severity Scale is easy to apply and will be useful as an outcome measure in treatment studies and enable the standardisation of other clinical and laboratory ENL research.

BackgroundErythema Nodosum Leprosum (ENL) is a serious complication of leprosy. It is normally treated with high dose steroids, but its recurrent nature leads to prolonged steroid usage and associated side effects. There is little evidence on the efficacy of alternative treatments for ENL, especially for patients who have become steroid resistant or have steroid side effects. These two pilot studies compare the efficacy and side effect profile of ciclosporin plus prednisolone against prednisolone alone in the treatment of patients with either new ENL or chronic and recurrent ENL.Methods and ResultsThirteen patients with new ENL and twenty patients with chronic ENL were recruited into two double-blinded randomised controlled trials. Patients were randomised to receive ciclosporin and prednisolone or prednisolone treatment only. Patients with acute ENL had a delay of 16 weeks in the occurrence of ENL flare-up episode, with less severe flare-ups and decreased requirements for additional prednisolone. Patients with chronic ENL on ciclosporin had the first episode of ENL flare-up 4 weeks earlier than those on prednisolone, as well as more severe ENL flare-ups requiring 2.5 times more additional prednisolone. Adverse events attributable to prednisolone were more common that those attributable to ciclosporin.ConclusionsThis is the first clinical trial on ENL management set in the African context, and also the first trial in leprosy to use patients’ assessment of outcomes. Patients on ciclosporin showed promising results in the management of acute ENL in this small pilot study. But ciclosporin, did not appear to have a significant steroid–sparing effects in patients with chronic ENL, which may have been due to the prolonged use of steroids in these patients in combination with a too rapid decrease of steroids in patients given ciclosporin. Further research is needed to determine whether the promising results of ciclosporin in acute ENL can be reproduced on a larger scale.

Background: Health-related quality of life (HRQoL) has now become an indispensable outcome measure in many randomized clinical trials and other studies. It provides the patient’s voice in measuring health improvement or decline and assessing treatment effectiveness. A validated Amharic version of HRQoL assessment tool was needed for leprosy clinical trials in Ethiopia. The SF-36 was chosen but a validated Amharic version was not available. We describe how this was developed.Methods: The SF-36 was translated from English into Amharic and evaluated for content acceptability in a patient focus group. Back translation was performed. Validity and reliability of Amharic SF-36 in leprosy affected individuals was tested with 100 patients with leprosy attending the leprosy clinic at ALERT hospital and compared to the Amharic version of the WHOQOL-BREF.Results: Amharic translations of both the WHOQOL-BREF and the SF-36 had good reliability and validity amongst leprosy affected individuals. Internal consistency reliability estimates for each domain/scale exceeded 0.70. The Amharic SF-36 had better convergent and discriminant validity than WHOQOL-BREF in this group of patients. Good known-group validity was seen in both WHOQOL-BREF and SF-36 in leprosy affected patients. Amharic SF-36 had good inter-rater reliability with seven out of 8 domains scoring above 0.8 in intra-class correlation.Conclusion: This Amharic version of the SF-36 is a valid instrument to measure HRQoL in clinical trials involving leprosy affected individuals in Ethiopia.

Case 1A 36 years old man presented to dermatology department, ALERT hospital for a consultation of his skin problem. He states that he has a congenital pigment disorder. He was worried if it were leprosy. He had no symptoms of leprosy such as numbness of the extremities or contact history either. The patient claimed the pigment disorder, mainly on his trunk, improved a lot over the years. He also brought two of his children (9yr old boy and 1yr and7/12 daughter) to the hospital, for they had similar congenital disorder. The children had no other medical problems.Physical examinations reveals poliosis (loss of pigment of the hair) over the scalp (fronto-temporal), medial part of the eye brow of the left side and the chin area. The skin over the anterior trunk has hypopigmented patches with areas of repigmentations and leukotrichia. There are also numerous discrete café-au-lait spots of different size (>15mm the largest one) located over the back of the patient. There were also axillary frecklings. No neurofibromas were observed (Figures1-3). Case 2Physical examination of the skin of the boy reveals patches of hypopigmentation and depigmentations over mid-forehead, chest, abdomen, upper and lower extremities. A white forelock and pigment loss of the medial eye brows were noted. Within the depigmented patches, islands of hyperpigmented macules are seen. Multiple hyperpigmented macules(café-au-lait spots of size >5 mm) were also seen over the back of this child .There were also axillary freckling. No neurofibromas were observed . AbstractPiebaldism is a rare disorder present at birth and inherited as an autosomal dominant trait. It results from a mutation in the c-kit proto-oncogene and is associated with a defect in the migration and differentiation of melanoblasts from the neural crest. Clinical manifestations and phenotypic severity strongly correlates with the site of mutation within the KIT gene. The white hair and patches of such patients are completely formed at birth and do not usually progress or regress thereafter.Here I report a family (one year and seven months old daughter, nine year old boy and their father) with piebaldism associated with clinical criteria for Neurofibromatosis type -1. There are rare reports of piebaldism associated with neurofibromatosis -1. I also report a case of piebaldism with regression of the depigmentation over the trunk. Regression of the white foreloke was rarely reported but regression over the depigmentation over the trunk has not been reported. Case 3Physical examination of the skin of the daughter reveals mild forelock, depigmented patch over the abdomen , multiple café-au-lait spots (>5 mm) on her back with axillary frecklings .