Purpura fulminans is a rare complication of a coagulopathy or an infection. Haemophilus influenzae infection, which has decreased since the haemophilus influenzae type B vaccine was initiated, is an unusual initiating cause of purpura fulminans. This case is the first reported in the literature of an adult who developed purpura ...

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are rare and closely related disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Many risk factors have been reported including infection, cancer, pregnancy, a variety of drugs (e.g. anticancer drugs), and autoimmune diseases. The incidence of TTP-HUS is higher ...

We report on a case of Henoch-Schönlein purpura (HSP) with pulmonary hemorrhage and severe renal involvement. The patient also had active carditis related to acute rheumatic fever. He died despite intensive treatment. Regarding this case, we discuss the pathogenesis and clinical findings of pulmonary hemorrhage and active carditis in HSP.

Pemphigoid nodularis is a chronic and uncommon variant of bullous pemphigoid. The condition is characterized by the presence of prurigo nodularis-like lesions, possible history of blistering, and immunohistochemical findings of bullous pemphigoid. These patients are often unresponsive to conventional therapy with potent topical and systemic steroids and different immunosuppressive agents ...

BACKGROUND/PURPOSE: The aim of this study was to present the current experience and evidence relating to surgical evaluation and management of nonrenal genitourinary presentation in children with Henoch-Schönlein purpura (HSP). Henoch-Schönlein purpura is the most common systemic vasculitis in childhood. Presenting symptoms include a rash, arthralgia, abdominal pain, and, frequently, ...

We present a case of a 53-year-old black male who developed a factor VIII inhibitor during his treatment for hepatitis C virus infection with peginterferon. The development of inhibitors against factor VIII during peginterferon therapy has been rarely reported. We have reviewed the current literature and the following case presentation ...

Henoch-Schonlein purpura (HSP) is the most common acute vasculitis in the pediatric population, with an incidence of 10-14 per 100,000. The classic presentation of this disorder includes erythematous papules followed by palpable purpura in the lower extremities, trunk, and face, arthralgia or arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. While ...

Although relatively rare, intracranial hemorrhage remains the most common cause of immune thrombocytopenic purpura-related mortality [Medeiros D. Current controversies in the management of idiopathic thrombocytopenic purpura during childhood. Pediatr Clin North Am . 1996;43:757-72]. The required decompressive treatment has the potential for substantial blood loss and must often be delayed ...

Several disease states manifest as thrombotic microangiopathies (TMA), most prominently thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). The recent discovery of the von Willebrand factor cleaving protease ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type 1 motif), found to be deficient in TTP, has helped separate these entities. ...

We reported the case of a child who died of purpura fulminans. The diagnosis of Neisseria meningitidis serogroup C could be assessed using postmortem specimens collected up to 10 h after death. We were able to identify the bacteria by culture and/or PCR on samples without having autopsy performed. Soluble ...

Thrombotic thrombocytopenic purpura (TTP), characterized by thrombocytopenia and microangiopathic hemolytic anemia, is a relatively rare disorder. The majority of cases have no defined causes. TTP has been reported in association with many drugs, but not with imatinib mesylate. We report a 22-year-old African-American woman who developed idiopathic hypereosinophilic syndrome. She ...

Intracranial haemorrhage (ICH) is a dramatic and potentially life-threatening presentation of children with thrombocytopenia. Management is limited to supportive care. Recent evidence suggests that ongoing bleeding following the initial ICH may result in greater neurological morbidity and mortality. Haemostatic agents, including recombinant factor VIIa (rFVIIa), a product licensed for use ...

A hallmark of the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) is evidence of microangiopathic hemolytic anemia. The presence of schistocytes on the peripheral blood smear, elevated plasma lactic dehydrogenase, and decreased haptoglobin concentration are used as evidence of microangiopathic hemolytic anemia to make a diagnosis of TTP. This report ...

Progressive pigmentary purpura is a rare condition characterized by lymphocytic capillaritis histologically causing various clinical entities which are also named as persistent pigmented purpuric dermatoses. It is generally idiopathic; however, rare cases secondary to drugs and various diseases have been reported. In this report we describe a case of progressive ...

Henoch-Schönlein purpura (HSP) is a common childhood vasculitis. Abdominal pain is a common feature of HSP, often leading to surgical consultation for evaluation of possible intussusception. Appendicitis is a rare complication of HSP, and in each of the 3 reported cases, appendectomy preceded the appearance of the purpuric rash. More ...

OBJECTIVES: To describe the association between thrombotic thrombocytopenic purpura (TTP) and systemic sclerosis (SSc) and the methods to distinguish TTP from scleroderma renal crisis (SRC). METHODS: A case of TTP that developed in a patient with preexisting SSc is described. Medline/PubMed was searched for literature pertaining to an association between ...

Thrombotic events in patients with inherited bleeding disorders occur only rarely. However, in some cases, the co-existence of acquired or inherited prothrombotic risk factors may overcome the hypocoagulative state, modulating the clinical phenotype to a decrease in bleeding symptoms or even to an increase in the likelihood of developing thrombotic ...

We report an agricultural fair-associated shiga-toxigenic Escherichia coli O157:H7 (STEC O157) outbreak that was unusual in that it affected both livestock exhibitors and visitors. Twenty-five human cases of STEC O157 infection were detected after the Fort Bend County Fair in Rosenberg, Texas, which ran from 9/26/03 to 10/04/03. Seven cases ...

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterised by platelet aggregation causing microvascular occlusion. Early diagnosis and utilization of plasmapheresis can provide an improvement in prognosis. CASE REPORT: A 17 year old male with classical findings of TTP was later diagnosed as systemic lupus erythematosus (SLE). Plasmapheresis resulted ...

Henoch Schonlein Purpura with acute rheumatic carditis is a rare entity and only few cases have been reported. An 8 year-old-girl presented with abdominal pain, arthralgia and rashes and was diagnosed as a case of Henoch Schonlein Purpura. She was managed conservatively and discharged. She was readmitted after 1 week ...

Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 has been demonstrated to be the proximate cause of a subset of thrombotic microangiopathic haemolytic anaemias (MAHA) typical for thrombotic thrombocytopenic purpura (TTP). ADAMTS13 gene mutations cause the hereditary form; acquired deficiency has been attributed to presence of an autoantibody, which ...

We present two brothers who came to us with similar complaints within a two-month interval and who were diagnosed as having Henoch-Schönlein purpura. Interestingly, the two brothers were prisoners in the same jail. In addition, we shall review the small number of familial Henoch-Schönlein purpura cases that have been reported ...

Thrombotic thrombocytopenic purpura, a rare clinical syndrome characterized by thrombocytopenia and microangiopathic hemolytic anemia, was almost uniformly fatal until the introduction of plasma exchange in 1970. The thrombotic thrombocytopenic purpura following cardiovascular surgeries is relatively a new entity with high mortality if untreated. Plasma exchange is the most important therapy ...

Breath-holding spells (BHS) are commonly seen in childhood. However, there are no case reports of BHS occurring in adolescents or young adults. We report two young adult cases and discuss the pathogensis, both physically and psychologically. BHS occurred for 1-2 minutes after hyperventilation accompanied by cyanosis in both cases. Oxygen ...

We report the case of a young healthy woman who presented an early overanticoagulation when receiving acenocoumarol for a first thromboembolic episode. The patient had none of the risk factors known to influence the response to the coumarinic derivative except that she carried the rare *3 allelic variant of the ...

A case report is presented of a naval aviator who suffered a transient ischemic attack (TIA) 1 mo after experiencing right leg pain and swelling following a 4-d drive. Although a venous thrombotic source was never discovered, the presence of both a patent foramen ovale and a thrombophilic condition supports ...

Thrombotic thrombocytopenic purpura is a rare but serious condition in childhood. It can be idiopathic or a complication of other diseases or drug therapy. We report on a 12-year-old Chinese girl who presented with fulminant systemic lupus erythematosus with progressive renal failure, pancytopenia, and cerebral dysfunction due to thrombotic thrombocytopenic ...

BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder. The pathogenesis is believed to be mediated by an autoantibody directed against the metalloproteinase responsible for the degradation of the very-high-molecular-weight multimers of the vWF. The syndrome can be precipitated by a variety of conditions, and certain medications also have ...

We present the case of a woman (age: 64 years) with acute thrombotic microangiopathy due to severe acquired ADAMTS-13 (von Willebrand factor-cleaving protease) deficiency. She was successfully treated with plasma exchange therapy and glucocorticosteroids. She relapsed seven months later, and splenectomy led to lasting remission. Pathomechanisms of thrombotic thrombocytopenic purpura, ...

Minocycline hydrochloride, a synthetic tetracycline, is a systemic antibiotic that has received much attention over the past several years. Currently, minocycline is considered the most widely prescribed oral antibiotic in the management of acne. Minocycline has been associated with autoimmune events, hepatitis, lupus-like syndromes, serum sickness, vasculitis, Sweet's syndrome, and ...

Bullous pemphigoid (BP) is an acquired bullous disorder that predominantly affects the elderly. It is rare in children but when it occurs, there is considerable clinical and histologic overlap with other acquired or congenital blistering disorders. A definitive diagnosis of childhood BP requires direct immunofluorescence and, in some cases, characterization ...

Although the antiplatelet agent clopidogrel is associated with an increased incidence of thrombotic thrombocytopenic purpura (TTP), available evidence is not sufficient to establish or disprove a causal relationship. We review and assess the evidence and case reports linking clopidogrel use with TTP and discuss how to approach the risk of ...

Osteopetrosis is a rare hereditary metabolic disease associated with a myriad of otolaryngological presentations. Three cases with varying otolaryngological presentations are described here. A brief review of the radiological changes of interest to the otorhinolaryngologist is being made.

Henoch-Schönlein purpura is one of the most common types of systemic vasculitis in children. Although recurrence is frequent, most cases are benign and self-limited. Standard treatment consists of supportive care and nonsteroid antiinflammatory drugs and/or steroid. Here we report 2 cases of Henoch-Schönlein purpura, both of which had a prolonged ...

Two cases of scurvy diagnosed following presentation with a purpuric rash are presented. A 44-year-old man developed scurvy as a result of poor dietary intake of vitamin C. This occurred because of a number of factors. including poor dentition, diarrhoea, depression and benzodiazepine/narcotic dependence. A 69-year-old man with acute myeloid ...

Bupropion hydrochloride is an effective drug for people who want stop smoking, and its use has recently increased in many countries. The main side effects of this drug are related to its dopaminergic activity and are dose dependent. To date, no cases of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) have ...

This article reports the unexpected discovery of IgA nephropathy in a 26-year-old Chinese woman 1.5 years after the onset of idiopathic dermatomyositis. The patient was taking immunosuppressive agents, prednisolone 25 mg and azathioprine 75 mg daily. Glomerulonephritis associated with idiopathic polymyositis/dermatomyositis is rare. A review of the medical literature indicates ...

Henoch-Schönlein purpura is an acute, self-limited vasculitis syndrome which shows characteristic skin, joint, renal and gastrointestinal manifestations. It is common in childhood and may also occur in adults with fatal complications such as nephritis and gastrointestinal bleeding. We experienced a case of a 20-year-old woman who presented with palpable purpura ...

Thrombotic thrombocytopenic purpura (TTP) is a rare disease. A high index of suspicion is required for early diagnosis and treatment. It can present in acute as well as chronic forms. Cyclophosphamide has been reported to be beneficial in relapsing TTP. Herein a case of acute TTP evolving into relapsing phase ...

Acute infectious purpura fulminans is a rapidly progressive syndrome of hemorrhagic skin necrosis associated with acute infection and disseminated intravascular coagulation. We report 5 cases of purpura fulminans and briefly review the literature. All cases were associated with encapsulated organisms (Streptococcus pneumoniae or Group A streptococcus), and 4 of the ...

Palmar petechiae or purpura is an unusual finding in dermatitis herpetiformis (DH) that occurs in children but is only rarely reported in adults. We describe a 46-year-old man with DH who presented with the classic pruritic papulovesicular eruption and associated volar finger and palmar petechiae. We discuss recent advances in ...

Pemphigoid gestationis (PG) is a rare dermal-epidermal autoimmune bullous disease of pregnancy and postpartum, which relapses more seriously and earlier during following pregnancies. PG also occurs in association with trophoblastic tumours or oral contraceptive treatment. The term 'persistent PG' represents the cases where active disease persists for months to many ...

Purpura fulminans (PPF) is a very severe but rare acute thrombohaemorrhagic illness of infants and young children. It occurs mainly, in patients with either congenital or acquired deficiencies of proteins C and S and antithrombin III. Features of PPF include disseminated intravascular coagulopathy, symmetrical necrotic purpura and/or ecchymoses and symmetrical ...

OBJECTIVE: To report the first case of ciprofloxacin-associated hemolytic-uremic syndrome (HUS). CASE SUMMARY: A 53-year-old white man was treated with chemotherapy for acute lymphoblastic leukemia. Four weeks after initiation of treatment, he recovered his blood cell counts, but developed fever and was prescribed oral ciprofloxacin 500 mg twice daily. After ...

We report on a 71-year-old male with Henoch-Schoenlein purpura (HSP) who developed glomerulocystic kidney disease (GCKD) without congenital abnormality. He had mild renal dysfunction. Renal biopsy findings showed mild proliferation of mesangial cells and matrixes, and tubular atrophy, interstitial fibrosis, cystic dilation of Bowman's capsule and atrophy of the glomerular ...

A healthy woman presented with ecchymoses due to thrombocytopenia, with numerous bone marrow megakaryocytes, microangiopathic haemolytic anaemia, disorientation, irritability, and normal renal function. Thrombotic thrombocytopenic purpura (TTP) was diagnosed and treated successfully by plasma exchange therapy, both on presentation and during a further three relapses. The TTP was considered idiopathic ...

The association of ulcerative colitis (UC) and thrombotic thrombocytopenic purpura (TTP) is rare. Only one prior patient with these two syndromes has been reported in the literature. In that case, splenectomy and proctectomy were performed to control the symptoms of TTP. We present two patients with UC who developed TTP ...

AIM: To describe a possible relationship between Henoch-Schönlein purpura and rheumatic fever. METHODS: Patients with features of both diseases were identified by reviewing the hospital records. Medline and reference lists from published articles were used to search for previous reports of the two conditions occuring simultaneously. RESULTS: Three newly described ...

Oral pemphigoid, defined as cicatricial pemphigoid limited to the oral cavity, is a clinical subset of cicatricial pemphigoid. Like cicatricial pemphigoid, it occurs mostly in middle-aged women and it is uncommon in children under 20 years of age. There are 11 cases of childhood cicatricial pemphigoid published previously in the ...

Pigmented purpuric eruptions comprise a group of benign dermatoses that are characterized clinically by pinpoint petechiae and purpura on a hyperpigmented base and histologically by capillaritis. The etiology of this group of disorders is unknown, although aberrant cell-mediated immunity has been proposed. Pigmented purpuric eruptions are well characterized in the ...