Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41- year-old woman who suffered fro...Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41- year-old woman who suffered from congenital afibrinogenaemia and hepatitis C viral infection and presented with ischaemic necrosis and livedo of the toes. Laboratory investigations showed the presence of mixed cryoglobulinaemia and anticardiolipin antibodies. Resolution occurred with plasmapheresis. We discuss the pathophysiology of this unusual condition and review the literature for skin manifestations associated with this rare haemostasis disorder.展开更多
Loxosceles spider bites are the only proven medically important cause of necrotic arachnidism in North America, and loxoscelism occurs far less commonly than is perceived by patients or physicians. In patients with ve...Loxosceles spider bites are the only proven medically important cause of necrotic arachnidism in North America, and loxoscelism occurs far less commonly than is perceived by patients or physicians. In patients with verified spider bites,an accurate diagnosis can be made only if the biting spider is identified by an experienced arachnologist An offending spider that is found in an area where loxosceles spiders are not endemic is most like lynotloxosceles, and necrosis is unlikely. In rare instances, bites from brown recluse spiders can cause clinically important dermal necrosis and subsequent scarring, but even severe necrosis is rarely life- threatening. Because of the tendency for medical reports to highlight noteworthy extreme cases, physicians may be unaware that the bite of a brown recluse spider is typically self-limited and self- healing, without long- term consequences.48 Patients often overemphasize spider involvement in idiopathic wounds, a tendency that can misdirect physicians toward an erroneous diagnosis. Physicians should be skeptical of any undocumented history of a spider bite and should entertain a broad differential diagnosis before attributing a skin ulcer to a spider bite. Misdiagnosis of an ulcer as loxoscelism delays proper treatment, placing the patient at risk. There is no therapy with proven efficacy for loxoscelism. Many questionable treatments have been tried in patients with an unverified diagnosis, and the medical literature on loxoscelism has been obfuscated by mis- diagnosed conditions. Both situations have inflated the spectrum of symptomatology, which may partly explain the confusion about therapeutic efficacy. Most practitioners would probably prescribe dapsone in patients with documented loxosceles bites, but even with this therapy, there is marginal evidence to support its use. Dapsone has potentially serious toxicity and should be prescribed judiciously. Other therapies, such as glucocorticoids, hyperbaric oxygen, and early excision, are also of unproven value. In questionable cases, the best approach may be the conservative use of simple first aid and local wound care. Recent advances in medical arachnology are resulting in a reassessment of how to approach patients with suspected necrotic spider bites. With refinement in the epidemiology of loxosceles bites and a greater understanding of the pathophysiology of necrosis, physicians are acquiring the tools to diagnose and treat loxoscelism more effectively.展开更多
Background: Necrotizing fascitis (NF) is a severe infection of the subcutaneous tissue and fascia affecting children and adults.Conventional management includes resuscitation, aggressive debridement of necrotic tissue...Background: Necrotizing fascitis (NF) is a severe infection of the subcutaneous tissue and fascia affecting children and adults.Conventional management includes resuscitation, aggressive debridement of necrotic tissue, and sometimes, additional measures such as hyperbaric oxygen and immunoglobulin therapy.This paper reports conservative management of 18 patients with NF with minimal morbidity and mortality. Material and Methods:Patients with NF admitted to our department between January 2000 and February 2004 were included in the study (N= 18). In all cases, the presentation was rapidly progressing cellulitis progressing to cutaneous gangrene between 6 and 18 hours. The patients were managed by aggressive fluid resuscitation,analgesia, broad-spectrum antibiotics, and dressing with liberal quantities of povidone iodine ointment. After separation of the gangrenous skin margins from the surrounding healthy tissue between 24 and 72 hours, dead skin and fascia were removed with forceps on the ward, the wound washed with liberal quantities of water, and the ointment dressing reapplied.This procedure was repeated until all the dead tissue had been removed. Once the wound was granulating, dressings were changed at increasing intervals until healing took place by secondary intention. Results: The patients were aged between 5 days and 11 years. In all, NF began as a small boil progressing to a rapidly spreading cellulitis. None of the patients was operated during the acute stage of the infection. Blackening of the skin and separation of the edges occurred within 8-72 hours,the dead tissue was allowed to separate from the granulating base and could be removed at the bedside with minimal blood loss. Blood transfusion was required only in 2 patients where hemoglobin was < 9 mg/dL. Of the 18 patients, 6 grew group A streptococci and staphylococci in a polymicrobial wound culture, whereas the other 12 had polymicrobial flora without streptococci. The clinical course and outcomes were similar in both types of wounds. There was 1 death in the study group, and1 patient required skin grafting. All other survivors had healing by secondary intention without disability. The period for complete epithelization varied between 3 and 8 weeks. Patients were discharged home when 70%of the wound had healed.There was extensive scarring in 3 children with NF involving the back. The other children had minimal or no scarring. None of the patients had any restriction in the movement of limbs or joints. These findings were compared with 16 retrospective patients of NF treated before January 2000 by the conventional approach of aggressive early debridement, the results of the conservative approach were superior with shorter hospital stay, lower number of blood transfusions, earlier appearance of granulation tissue, and shorter duration of complete healing.Conclusions: We conclude that the conservative management of NF offers advantages in morbidity without compromising the outcome. In our hospital setup, conservative treatment was less expensive and easily carried out. We would therefore advocate conservative management for the treatment of this condition.展开更多
Background. At the beginning the antiphospholipid antibodies syndrome was associated with systemic lupus erythematosus. But since 1988 it has become a sole entity. Its current definition is based on the criteria estab...Background. At the beginning the antiphospholipid antibodies syndrome was associated with systemic lupus erythematosus. But since 1988 it has become a sole entity. Its current definition is based on the criteria established in 1999 by Sapporo and consists of associating the clinical criteria of thrombosis of arteries or peripheral veins and of miscarriage of pregnancy with the biological criteria. Either anti-cardiolipin antibodies or lupus anticoagulant must be present. Anti-phosphatidylethanolamine antibodies are not included in the Sapporo criteria. Case report. A non smoking, 43 year-old man showed a clinical manifestation of livedo on the thighs, and left knee and foot, associated with a rapidly extending cutaneous necrosis on the left toes. One year earlier his right leg was amputated up to half of the calf following distal gangrene. The gangrene was consecutive to a stent implantation after a significant stenosis of the right superficial femoral artery. The etiological investigations revealed neither thrombophily nor cholesterol embolism nor vasculitis. No sign of underlying neoplasia could be found. These clinical symptoms as well as the anamnesis were strongly suggestive of an antiphospholipid antibodies syndrome. The immunological dosages revealed isolated positive anti-phosphatidylethanolamine antibodies,persistent six weeks later. Discussion. Several cases of clinical manifestations of the antiphospho lipid antibodies syndrome have been described, without any anti-cardiolipin antibodies or lupus anticoagulant, but with presence of anti-phosphatidylethanolamine antibodies. In cases of these strong evocative symptoms but no evidence of the classical biological Sapporo criteria,these antibodies should be systematically searched for.展开更多
文摘Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41- year-old woman who suffered from congenital afibrinogenaemia and hepatitis C viral infection and presented with ischaemic necrosis and livedo of the toes. Laboratory investigations showed the presence of mixed cryoglobulinaemia and anticardiolipin antibodies. Resolution occurred with plasmapheresis. We discuss the pathophysiology of this unusual condition and review the literature for skin manifestations associated with this rare haemostasis disorder.
文摘Loxosceles spider bites are the only proven medically important cause of necrotic arachnidism in North America, and loxoscelism occurs far less commonly than is perceived by patients or physicians. In patients with verified spider bites,an accurate diagnosis can be made only if the biting spider is identified by an experienced arachnologist An offending spider that is found in an area where loxosceles spiders are not endemic is most like lynotloxosceles, and necrosis is unlikely. In rare instances, bites from brown recluse spiders can cause clinically important dermal necrosis and subsequent scarring, but even severe necrosis is rarely life- threatening. Because of the tendency for medical reports to highlight noteworthy extreme cases, physicians may be unaware that the bite of a brown recluse spider is typically self-limited and self- healing, without long- term consequences.48 Patients often overemphasize spider involvement in idiopathic wounds, a tendency that can misdirect physicians toward an erroneous diagnosis. Physicians should be skeptical of any undocumented history of a spider bite and should entertain a broad differential diagnosis before attributing a skin ulcer to a spider bite. Misdiagnosis of an ulcer as loxoscelism delays proper treatment, placing the patient at risk. There is no therapy with proven efficacy for loxoscelism. Many questionable treatments have been tried in patients with an unverified diagnosis, and the medical literature on loxoscelism has been obfuscated by mis- diagnosed conditions. Both situations have inflated the spectrum of symptomatology, which may partly explain the confusion about therapeutic efficacy. Most practitioners would probably prescribe dapsone in patients with documented loxosceles bites, but even with this therapy, there is marginal evidence to support its use. Dapsone has potentially serious toxicity and should be prescribed judiciously. Other therapies, such as glucocorticoids, hyperbaric oxygen, and early excision, are also of unproven value. In questionable cases, the best approach may be the conservative use of simple first aid and local wound care. Recent advances in medical arachnology are resulting in a reassessment of how to approach patients with suspected necrotic spider bites. With refinement in the epidemiology of loxosceles bites and a greater understanding of the pathophysiology of necrosis, physicians are acquiring the tools to diagnose and treat loxoscelism more effectively.
文摘Background: Necrotizing fascitis (NF) is a severe infection of the subcutaneous tissue and fascia affecting children and adults.Conventional management includes resuscitation, aggressive debridement of necrotic tissue, and sometimes, additional measures such as hyperbaric oxygen and immunoglobulin therapy.This paper reports conservative management of 18 patients with NF with minimal morbidity and mortality. Material and Methods:Patients with NF admitted to our department between January 2000 and February 2004 were included in the study (N= 18). In all cases, the presentation was rapidly progressing cellulitis progressing to cutaneous gangrene between 6 and 18 hours. The patients were managed by aggressive fluid resuscitation,analgesia, broad-spectrum antibiotics, and dressing with liberal quantities of povidone iodine ointment. After separation of the gangrenous skin margins from the surrounding healthy tissue between 24 and 72 hours, dead skin and fascia were removed with forceps on the ward, the wound washed with liberal quantities of water, and the ointment dressing reapplied.This procedure was repeated until all the dead tissue had been removed. Once the wound was granulating, dressings were changed at increasing intervals until healing took place by secondary intention. Results: The patients were aged between 5 days and 11 years. In all, NF began as a small boil progressing to a rapidly spreading cellulitis. None of the patients was operated during the acute stage of the infection. Blackening of the skin and separation of the edges occurred within 8-72 hours,the dead tissue was allowed to separate from the granulating base and could be removed at the bedside with minimal blood loss. Blood transfusion was required only in 2 patients where hemoglobin was < 9 mg/dL. Of the 18 patients, 6 grew group A streptococci and staphylococci in a polymicrobial wound culture, whereas the other 12 had polymicrobial flora without streptococci. The clinical course and outcomes were similar in both types of wounds. There was 1 death in the study group, and1 patient required skin grafting. All other survivors had healing by secondary intention without disability. The period for complete epithelization varied between 3 and 8 weeks. Patients were discharged home when 70%of the wound had healed.There was extensive scarring in 3 children with NF involving the back. The other children had minimal or no scarring. None of the patients had any restriction in the movement of limbs or joints. These findings were compared with 16 retrospective patients of NF treated before January 2000 by the conventional approach of aggressive early debridement, the results of the conservative approach were superior with shorter hospital stay, lower number of blood transfusions, earlier appearance of granulation tissue, and shorter duration of complete healing.Conclusions: We conclude that the conservative management of NF offers advantages in morbidity without compromising the outcome. In our hospital setup, conservative treatment was less expensive and easily carried out. We would therefore advocate conservative management for the treatment of this condition.
文摘Background. At the beginning the antiphospholipid antibodies syndrome was associated with systemic lupus erythematosus. But since 1988 it has become a sole entity. Its current definition is based on the criteria established in 1999 by Sapporo and consists of associating the clinical criteria of thrombosis of arteries or peripheral veins and of miscarriage of pregnancy with the biological criteria. Either anti-cardiolipin antibodies or lupus anticoagulant must be present. Anti-phosphatidylethanolamine antibodies are not included in the Sapporo criteria. Case report. A non smoking, 43 year-old man showed a clinical manifestation of livedo on the thighs, and left knee and foot, associated with a rapidly extending cutaneous necrosis on the left toes. One year earlier his right leg was amputated up to half of the calf following distal gangrene. The gangrene was consecutive to a stent implantation after a significant stenosis of the right superficial femoral artery. The etiological investigations revealed neither thrombophily nor cholesterol embolism nor vasculitis. No sign of underlying neoplasia could be found. These clinical symptoms as well as the anamnesis were strongly suggestive of an antiphospholipid antibodies syndrome. The immunological dosages revealed isolated positive anti-phosphatidylethanolamine antibodies,persistent six weeks later. Discussion. Several cases of clinical manifestations of the antiphospho lipid antibodies syndrome have been described, without any anti-cardiolipin antibodies or lupus anticoagulant, but with presence of anti-phosphatidylethanolamine antibodies. In cases of these strong evocative symptoms but no evidence of the classical biological Sapporo criteria,these antibodies should be systematically searched for.
