1/9. Tropical idiopathic lower limb gangrene: case report.Tropical idiopathic lower limb gangrene is a rare disease. It was first described by Gelfand amongst the indigenous inhabitants of present day zimbabwe. It is a bilateral and simultaneous gangrene of both lower extremities due to no obvious cause and usually seen in men during the second and fourth decade of life. The onset is always sudden and the first sign is oedema of both feet accompanied by pain. The patients are usually people who have been previously healthy. This is a report of a clinical variant of the disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. Electromyographic mixed nerve and cutaneous silent period in evaluating the A-delta fibres in a patient with hereditary sensory-autonomic neuropathy.The aim of this study was to evaluate A-delta fibre function in a patient with hereditary sensory-autonomic neuropathy (HSAN). We used the mixed and cutaneous silent period techniques in addition to a conventional electromyographic investigation in a patient with type 2 HSAN, a rare disease characterised by wide-spread sensory and variable autonomic dysfunction caused by incomplete development of sensory and autonomic neurons. Whereas the stimulation of one digital nerve did not show any evidence of silent period in either the left or the right hand, the simultaneous stimulation of two digital nerves, as well as the stimulation of a mixed nerve, revealed a measurable delayed and shortened silent period. These data suggest that a spatial summation mediated by A-delta fibres was required for generation of the silent period in this patient and that combining the CSP and MNSP may be of practical use in evaluating impairment of the small myelinated fibres.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. Management of cutaneous manifestations of extensive purpura fulminans in a burn unit.purpura fulminans is a rare disease, which may have devastating cutaneous manifestations. It usually follows an infectious illness, and although it most commonly occurs in children, it can occur in adults. The pathogenesis may be related to a relative deficiency of protein C and/or protein s. A case of an adult male is presented to illustrate the management of the severe full-thickness skin loss and the use of surgical excision and allograft in this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. Cryofibrinogenemia: report of a case.Cryofibrinogenemia, a rare disease, is diagnosed by the presence of reversible cryoprecipitate in plasma, which may cause various clinical manifestations of thromboembolic or hemorrhagic disorders. A 66-year-old female patient suffered from recurrent reticulate erythema, purpura and gangrene on the distal portion of the extremities and face during the winter for 5 years. Cryofibrinogenemia was proven with the following evidences: (1) Histopathologic findings revealed many eosinophilic cylinders, about 6-9 microns in diameter, deposited within the lumen of the blood vessels in the dermis and subcutaneous tissue. There was no evidence of vasculitis. These cylinders were fibrinogen in nature, as proven by direct immunofluorescence. (2) Cryofibrinogen was detected in the citrated plasma, which was confirmed by double immunodiffusion, the presence of an IgG-kappa M protein was also revealed by immunofixation. debridement and skin grafts were performed, and prednisolone combined with cyclophosphamide were given. skin lesions improved without recurrence, and the "cryocrit" decreased from 13.6% to less than 2% during the follow up. We conclude that if there is any case with the similar cutaneous manifestations, cryofibrinogenemia should be considered.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. Juvenile gangrenous vasculitis of the scrotum: Is it a variant of pyoderma gangrenosum?Juvenile gangrenous vasculitis of the scrotum was described by Pinol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acute onset of skin ulcers undergoing complete resolution after appropriate therapy, with no relapses. We present a typical case of this extremely rare disease affecting a 16-year-old boy in whom the scrotal ulcerations were preceded by an episode of pharyngitis with fever. The condition promptly regressed after administration of intramuscular betamethasone in combination with oral ciprofloxacin. According to Pinol et al, juvenile gangrenous vasculitis of the scrotum, although poorly known to dermatologists, should be regarded as a distinctive entity within the wide group of scrotal gangrenes. On the other hand, the hypothesis that this condition may well represent a variant of pyoderma gangrenosum is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Venous gangrene of the limb. Pathophysiological and therapeutic considerations.Venous gangrene of the limb is a rare disease. Peripheral arterial collapse has been shown to underlie the ischaemia. The two patients studied had venous gangrene and malignancy of the gastrointestinal tract with liver metastases. One patient had slowly and the other rapidly progressing gangrene despite heparin-warfarin therapy. Both patients died. microscopy revealed occlusive thrombosis of small peripheral arteries of the leg. The authors conclude that the pathophysiology in venous gangrene may be occlusive thrombosis of small arteries. This situation is the lethal form of the entity and responds poorly to established therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. purpura fulminans secondary to xanthomonas maltophilia sepsis in an adult with aplastic anemia.purpura fulminans is a rare disease characterized by purpura ecchymosis, hypotension, and fever associated with disseminated intravascular coagulation. It often begins as a benign infectious process and subsequently progresses to a severe, catastrophic outcome. It is recognized to originate from congenital or acquired protein c deficiency. We present an unusual case of an adult with xanthomonas maltophilia sepsis that subsequently developed into purpura fulminans with involvement of the four extremities. We discuss the importance of the protein C system in coagulation homeostasis and its relationship to purpura fulminans.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. purpura fulminans.purpura fulminans is a rare disease that typically begins as a benign infectious process and subsequently progresses to severe sepsis, hypotension, purpura ecchymosis, and disseminated intravascular coagulation. We present an unusual case of an adult who was seen initially with pneumococcal sepsis that subsequently developed into purpura fulminans with major extremity involvement. A multidisciplinary approach is needed in the treatment of this often catastrophic disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/9. Fournier's gangrene of the penis: a report of two cases.Fournier's gangrene is a rare disease involving the scrotum and penis with occasional extension up the abdominal wall. Both of our cases were unusual in that only the penis was involved. In addition, the occurrence of squamous cell carcinoma in an area previously affected by Fournier's gangrene has never been reported. The usual organism is an anaerobic streptococcus synergistic with some second organism. Early therapy is key, including debridement of the entire shaft of the penis distal to the devastated area, intravenous broad-spectrum antibiotics, and skin grafting.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |