RESUMO
Introduction: Bullous pemphigoid (BP) is considered the most common bullous autoimmune disorder, characterized by autoantibodies directed against hemidesmosomes in the skin and mucous membranes. It usually affects elderly individuals in the sixth through eighth decades of life, with an average age at onset of 65 years. Only a few cases have been reported in children and teenagers. Case presentation: Herein, we report a 17-year-old boy who presented with a pruritic vesicular rash on his arms and legs accompanied by erythema. He was treated at the beginning with topical lotion and acyclovir, but the rash kept deteriorating and eventually bullae appeared, involving also his mouth. A dermatologist was consulted and diagnosed him with BP, and he was treated accordingly. Discussion: BP is the most prevalent autoimmune bullous illness, caused by autoantibodies against hemidesmosomes in the basement membrane of skin and mucosal surfaces, which in turn attract immune cells, including T-cells and neutrophils, and activate them, which causes damage to and separation of keratinocytes, resulting in the bullous formation. Diagnosis can be accomplished by recognizing clinical symptoms supported by histopathological and immunofluorescence testing. Steroids, whether topical or systemic, are the cornerstone treatment; depending on the extent of the disease, other immunosuppressant drugs can be used as a second line. Conclusion: BP manifestations are polymorphic; physicians should keep in mind that they may present with non-bullous, pruritic lesions, which may persist for some days to several months before bullae appear. Although this disease is rare in the young population, it should be considered in the differential diagnosis of bullous lesions.
RESUMO
BACKGROUND: In 2013, al-Shifa Hospital, the largest hospital in the Gaza Strip, introduced an assessment programme for potential kidney donors on the basis of medical and radiological evaluation. The aims of this study and an associated clinical audit were to examine the final outcome of the potential live kidney donors assessment programme, to review the findings and determine the level of accuracy of the radiological evaluations, to assess the completeness and standard of radiological record keeping, and to evaluate adherence to guidelines. METHODS: Donor assessment files were retrieved from the kidney transplant database in the renal department of al-Shifa Hospital. Patients who had surgery outside the Gaza Strip were excluded. Delivery of the assessment programme was assessed according to British Transplantation Society guidelines. Radiological investigations were reviewed. The head of the radiological department was interviewed to provide additional information about the approach to assessing donors and current challenges. FINDINGS: We retrieved files for 35 potential live kidney donors (mean age 36·82 years [range 23-54]). 32 (91%) donors had a nephrectomy, 33 (94%) donors were directed donors, and the type of donor donation was unknown for two (6%) donors. 24 (69%) donors were women, and 11 (31%) were men. 24 (69%) donors had CT angiography imaging reports as part of their pre-surgical evaluation. Digital access to those reports was limited and inefficient. Radiological assessments of renal parenchymal and renal anatomy and variants were made in 96% of cases. Of the 24 donors whose CT angiography imaging reports were retrieved, the excretory system was assessed in 16 (67%) donors, the presence of nephrolithiasis was assessed in 12 (50%) donors, and parenchymal disease was found in one (4%) donor. INTERPRETATION: The potential live kidney donors assessment programme requires improvement to achieve an organised, consistent, and comprehensive radiological approach to assist clinical decisions, research, and quality assurance. We recommend using the latest radiology management system for an efficient workflow that can provide economical storage and convenient and timely access to images, interpretation, and digital transmission (eg, the Picture Archiving and Communication system). FUNDING: None.
