THE title Morbus Maculosis Werlhofii for the severe form of purpura haemorrhagica is derived from the fact that the first clear and accurate clinical account of this condition was made by Werlhof in 1775. However, over two centuries earlier, a Portuguese called Amatus Lusitanus described in one of his works, under the title of Morbus Pulicaris Absque Febre, the case of a boy with undoubted acut...

Henoch Schonlein purpura is a systemic vasculitis that commonly affects children and teenagers but also affects adults of all ages. In most instances it has a benign course. Organ involvement, particularly in adults, and notably the kidneys and gastrointestinal tract may require therapeutic intervention and may have a less favorable outcome. We report a case of a 58-year-old man who presented w...

CONTEXT Pancreatic adenocarcinoma presenting as idiopathic thrombocytopenic purpura has not been previously reported in the literature. CASE REPORT A newly diagnosed patient with metastatic pancreatic adenocarcinoma developed thrombocytopenic purpura prior to initiation of chemotherapy. Generalized petechiae were present and peripheral smear revealed giant platelets. Drug induced and other th...

Henoch Shonlein purpura (HSP) is a form of systemic vasculitis characterized by deposition of IgA dominant immune complexes in the small vessels. The triad of palpable purpuric rash on lower extremities, abdominal or renal involvement and arthritis is the typical presentation in this condition. The disease primarily affects children and is less common in adults. We report a case of a young fema...

Acute myocardial infarction (AMI) in patients with immune thrombocytopenic purpura (ITP) is rare. We describe a case of AMI in patient with ITP. An 81-year-old woman presented with acute inferoposterior MI with low platelet count on admission (34,000/µl). Coronary angiography revealed significant mid right coronary artery (RCA) stenosis with thrombus, subsequently underwent successful percutane...

A 63-year-old woman with abdominal pain and melena developed a palpable, purpuric rash and acute kidney injury. Skin and kidney biopsy confirmed Henoch-Schönlein purpura. Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus. AEN is a very rare cause of gastrointestinal hemorrhage...

A 27 year old housewife developed thrombotic thrombocytopenic purpura during the twelfth week of pregnancy. She had partial response to initial plasma infusion and subsequent plasmapheresis. However, her clinical course was complicated by the development of severe pancytopenia the consequence of a hypocellular marrow. She succumbed to septicaemic shock one month after diagnosis. The development...

We present a 12-year-old girl with systemic lupus erythematosus and associated antiphospholipid syndrome who developed an unusual manifestation of purpura fulminans in an accelerated fashion. The patient improved after prompt treatment with anticoagulants, aggressive immunosuppressive drugs and plasmapheresis. This is the first pediatric case of purpura fulminans due to secondary antiphospholip...

We describe a bilateral leukocoria and neonatal purpura fulminans in a male infant, born at full term after an unremarkable pregnancy to a healthy consanguineous married couple. Multiple hemorrhagic skin bullae were found at birth on various parts of the body with bilateral leukocoria, organized vitreous hemorrhage, retinal detachment, and intracranial hemorrhage with undetectable levels of pro...

how to cite this article: shiari r, tabatabaei nodushan smh, mohebbi mm, karimzadeh p, javadzadeh m. moyamoya syndrome associated with henoch-schönlein purpura. iran j child neurol. autumn 2016; 10(4):71-74. abstract some reports have shown the association between moyamoya syndrome and autoimmune diseases. herewith, we present a 3.5 yr old girl with henoch- schönleinpurpura (hsp) who was treate...