1/7. Ophthalmic manifestations of neonatal onset multisystem inflammatory disease.PURPOSE: To report the ophthalmic manifestations of neonatal onset multisystem inflammatory disease, a recently recognized, rare systemic disorder characterized by the triad of arthropathy, rash, and abnormal central nervous system development. METHOD: Case report. RESULTS: A 2-year-old female with neonatal onset multisystem inflammatory disease presented with visual acuity of fix and follow with each eye, bilateral optic nerve head pallor and gliosis, as well as marked sheathing of the peripapillary vessels. No vitreous inflammation or macular edema was found. visual acuity was stable from the neonatal period through the 3 months of follow-up after the changes involving the optic nerve heads and peripapillary vessels were observed (a total of 33 months). CONCLUSIONS: This report provides a description of the ocular manifestations of neonatal onset multisystem inflammatory disease and supports earlier suggestions that swelling of the optic nerve heads can occur. In this case, optic nerve head pallor may have been a sequela of such swelling. The pathogenesis of neonatal onset multisystem inflammatory disease is unknown.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/7. Atypical presentation of churg-strauss syndrome: another "forme fruste" of the disease?vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels. A broad and heterogenous group of syndromes may result from this process, because any type, size, and location of blood vessel may be involved. The cause of these conditions remains unclear, but an autoimmune inflammatory process, characterized by involvement of both neutrophils and endothelial cells, seems to play an important role. In 1951, Churg and Strauss described a clinical syndrome of severe asthma, hypereosinophilia with eosinophilic infiltrates, eosinophilic vasculitis, and granulomata in various organs. asthma may precede this vasculitis by many years. We report a case of anti-neutrophil cytoplasmic antibody-positive, pauci-immune, crescentic, necrotizing glomerulonephritis with peripheral and interstitial eosinophilia but without asthma. This is very unusual in churg-strauss syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/7. Two men with toxic shock syndrome presenting with targetoid and spotty skin rashes.Two previously healthy men who presented with hypotension, constitutional symptoms, and targetoid and discrete spotty erythematous plaques were diagnosed with toxic shock syndrome based on histopathological findings. Specifically, their biopsies revealed necrotic keratinocytes, neutrophils in the epidermis, and neutrophils surrounding dilated superficial vessels. In one case, the diagnosis of toxic shock syndrome was confirmed with rising titers to toxic shock syndrome toxin-1. Both patients recovered with supportive care and clindamycin administration. We suggest that patients with fever, hypotension, constitutional symptoms and rash should be started on clindamycin and have a skin biopsy as part of their initial evaluation. An understanding that toxic shock syndrome can strike anyone has manifold dermatological manifestations and defined histopathological findings is important for its early diagnosis and effective treatment.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
4/7. Successful treatment of mesenteric vasculitis caused by Henoch-Schonlein purpura with methylprednisolone pulse therapy.Although mesenteric vasculitis due to Henoch-Schonlein purpura (HSP) is relatively uncommon, it is the most life-threatening manifestation associated with high mortality. We describe a 15-year-old boy with HSP who had massive gastrointestinal bleeding and ileus but delayed onset of the purpuric rash. Abdominal ultrasonography revealed thickening of both small and large intestinal walls, and CT found prominent mesenteric vessels with comb sign and double wall of the bowel. These findings were consistent with mesenteric vasculitis and bowel ischaemia. The ischaemic intestine recovered after methylprednisolone pulse therapy and surgical intervention was avoided. Our report suggests that corticosteroid pulse therapy may help controlling HSP with massive gastrointestinal haemorrhage and ischaemic bowel due to widespread mesenteric vasculitis.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/7. Fatal fulminant hypereosinophilic syndrome with a petechial rash in a 16-year-old girl.Peripheral blood eosinophilia and eosinophilic tissue infiltration characterize the hypereosinophilic syndrome, which if untreated can be fatal. Its manifestations are protean. Often a diagnosis of exclusion, it presents diagnostic challenges. A fatal case of hypereosinophilic syndrome in a 16-year-old girl with petechiae, edema, urticaria, and diffuse erythema unfolded over 2 weeks. The histopathology of the petechiae demonstrated an eosinophilic and mixed cell interstitial and perivascular infiltrate and microthrombi in vessels. There was no evidence of T-cell clonality. Despite treatment with cyclosporine and methylprednisolone, she died. The age group; fulminant, fatal course without neoplasia; striking eosinophilia; and petechial rash are notable.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
6/7. A case of symptomatic primary HIV infection.A 30-year-old homosexual Japanese man had fourteen days of fever, malaise, appetite loss, sore throat, and four days of diarrhea and slightly congested eyes before he developed a skin eruption. He presented with measles-like exanthems on his face, trunk, and extremities. Deep red enanthems were seen on his left buccal mucosa opposite the premolar teeth, and whitish enanthems were seen on the buccal and gingival mucosa. HIV rna was detected at the high concentration of 5.8 x 10(6) copies /ml in his serum. cerebrospinal fluid examination revealed aseptic meningitis with 5,488 copies /ml of HIV rna. Anti-HIV 1 antibodies against Gp160 and p24 tested by Western blot assay showed seroconversion on day 5 of his admission, seven days after he developed the skin eruptions. The fever lasted for three weeks from the initial onset, and the skin eruptions lasted for twelve days. Histopathologically, a mononuclear cell infiltration was seen mainly in the upper dermis surrounding small vessels and sweat ducts, with CD8 cytotoxic T lymphocytes predominant. Additionally, CD1a putative interdigitating dendritic cells had also infiltrated perivascularly, and were surrounded by CD8 and CD4 T cells. in situ hybridization study failed to detect HIV products in skin biopsy specimens. Our findings suggested that CD8 T cells and their interaction with CD1a dendritic cells in the skin may be important in inducing skin manifestations in acute hiv infections.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
7/7. Eruptive pseudoangiomatosis.Eruptive pseudoangiomatosis (EPA) is a rare, self-limiting exanthem, which is known to occur primarily in children. It is characterized by an eruption of distinctive erythematous angioma-like papules often surrounded by a pale halo, with histological findings distinct from that of true angiomas. We describe three women with angioma-like papules. The biopsy specimens from them showed a unique histological appearance consisting of dilated dermal blood vessels with plump endothelial cell and perivascular lymphocytes. On the basis of the clinical and histological findings, we present the adult cases of eruptive pseudoangiomatosis and their etiology.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |