1/20. Fulminant and relentless cutaneous necrosis with excruciating pain caused by calciphylaxis developing in a patient undergoing peritoneal dialysis.A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/20. Primary uterine lymphoma: a case report.Primary lymphoma of the uterus is a rare disease, the reported characteristic MR imaging findings being homogeneous intermediate signal intensity of the indistinct mass on T1- and T2-weighted images, and the preservation of endometrial lining and uterine architecture. We report a case of primary uterine lymphoma which showed tumoral necrosis, endometrial disruption and diffuse anterior vaginal wall involvement.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/20. Necrotising fasciitis in the head and neck region.Necrotising fasciitis is an uncommon entity in present day medicine. Our review of the literature did not reveal any case involving the head and neck region. A case of this rare disease involving the head and neck region is presented.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/20. 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) |
5/20. Intraoral actinomycotic lesion: a case report.actinomycosis often referred to, as the chameleon of the head and neck pathology is a rare disease, uncommon in children. This article refers to a case of intraoral actinomycotic lesion of the palate in a child following a rare aetiology, the relevant literature, clinical course and its successful resolution.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/20. Malignant angioendotheliomatosis mimicking systemic necrotizing vasculitis.Malignant angioendotheliomatosis is a rare disease characterized by an intravascular proliferation of atypical mononuclear cells. Manifestations result from occlusion of small blood vessels. Multiple organ systems are involved and the clinical presentation resembles a systemic necrotizing vasculitis with skin and central nervous system most commonly involved. The clinical course is characterized by progressive organ failure with death usually within 2 years after presentation. Based on its assumed origin as an intravascular lymphoma, patients may respond to chemotherapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/20. Cutaneous anthrax on eyelids.BACKGROUND: Ophthalmologists should be aware of the signs and symptoms of anthrax, although it is a rare disease in humans. We report our successful treatment of three patients with cutaneous lesions in the periorbital area. CASES: In this study, we report on the treatment of three female patients who were initially diagnosed as having preseptal cellulitis. OBSERVATIONS: Gram-positive robs were revealed in the microscopic examination of scrapings taken from the lesions. bacillus anthracis was found in only two of the three scraping-material cultures. Intravenous penicillin g was administered in all cases. Black and necrotic eschar, which is characteristic of anthrax, developed on the eyelids of all three patients during treatment. At the final examinations of the patients after the completion of treatment, we recognized the development of cicatrisation, lagophthalmos, and slight ectropion in the upper eyelid of the first patient, and, in the second patient, restriction of upper eyelid movement and development of a corneal scar from exposure keratopathy and ectropion. The cutaneous lesions healed without any eyelid pathology in the third patient. CONCLUSION: Although it is a rare disease in humans, anthrax should be considered in the differential diagnosis of preseptal and orbital cellulitis.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
8/20. Bilateral extensive vascular calcification of the breast associated with coagulative necrosis: a calciphylaxis-like syndrome.A case of asynchronous bilateral breast necrosis in a 40-year-old woman with untreated chronic hypercholesteraemia is described. Mastectomies were performed, and histological examination revealed bilateral breast calciphylaxis characterized by extensive vascular calcification with coagulative necrosis. breast calciphylaxis is a rare disease clinically characterized by progressive tissular necrosis with secondary cutaneous ulceration and by vascular calcification and thrombosis. However, the nature of the vascular calcification has remained poorly understood up to now, owing to the absence of any precise mineralogical examination. In this case the mineral deposits were analysed for the first time: only hydroxyapatite was found. The etiology is discussed, and the high cholesterol rate of the patient is implicated.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/20. Cladophialophora bantiana brain abscess in a solid-organ transplant recipient: case report and review of the literature.cerebral phaeohyphomycosis caused by Cladophialophora bantiana is a rare disease. We describe a heart and bilateral lung transplant recipient who was unsuccessfully treated for a C. bantiana brain abscess. This report compares the present case to those of other solid-organ transplant recipients with the same infection and to those of patients who did not receive transplants.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/20. Torsion of the hernia sac within a hydrocele of the scrotum in a child.Torsion of the hernia sac is a rare disease that presents as acute scrotum in children. Including the present case, only six cases have been reported in the English literature. We report a 10-year-old boy who presented with pain and swelling of his right scrotum. ultrasonography revealed a hypoechoic region adjacent to the normal right testis. The inflammatory changes of the right scrotum deteriorated. The patient underwent surgery and a necrotic cyst was recognized within a hydrocele of the scrotum. The cyst was not connected with the testis or epididymis and was twisted at an angle of 270 degrees. The cause of the necrotic cyst observed was anatomical and pathological torsion of the hernia sac.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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