Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome

Urticarial vasculitis is a clinicopathologic entity in which episodes of urticaria are accompanied by histopathologic features of cutaneous vasculitis. The histopathologic definition of vasculitis varies from report to report. In this article, vasculitis is defined as histopathologic features of blood vessel damage: There should be evidence of leukocytoclasis and vessel wall destruction, which may or may not be accompanied by fibrinoid deposits. Red blood cell extravasation and perivascular inflammatory cell infiltrate also may be present. The extent to which each of these elements must be present has been debated.     

EBNA1 expression in a lung transplant recipient with hypocomplementemic urticarial vasculitis syndrome

This article describes a transplant recipient with underlying hypocomplementemic urticarial vasculitis syndrome who expressed persistently Epstein-Barr virus nuclear antigen 1 (EBNA1) in peripheral blood. The patient received a bilateral lung transplant and was subsequently followed with monitoring of EBV expression in peripheral blood. Evaluation of viral expression in peripheral blood, serum, and graft tissue was performed with RT-PCR, Q-PCR, indirect immunofluorescence, anti-peptide assays, and in situ hybridization; samples were collected at various time-points up to 91 days post-transplantation. The patient expressed EBNA1 in 8/10 (80%) of the peripheral blood samples tested during the post-transplantation period, and interestingly, even including the day of transplantation. After analyses of indicative EBV mRNA, EBNA1 expression was found mainly to be Qp-initiated EBNA1, known to be important for EBV maintenance. Anti-EBNA1 epitope mapping showed significantly higher and broader antibody responses to EBNA1 epitopes pre-transplantation when compared to normal controls and a matched lung transplant control. Post-transplantation this response was largely diminished but there were still epitopes significantly higher than controls. Our results show the presence of EBV-positive proliferating cells before onset of intensive immunosuppressive treatment. Although no previous connection between EBV and hypocomplementemic urticarial vasculitis syndrome has been reported, it is tempting to speculate that the continuous EBNA1 expression is not caused by immunosuppression or post-transplant lymphoproliferative disease, but may be a factor involved in the etiology of the autoimmune disease.     

Diffuse large B cell lymphoma in a patient with hypocomplementemic urticarial vasculitis

Hypocomplementemic urticarial vasculitis (HUV) is known to be associated with malignancies. Urticarial vasculitis has been linked to lymphomas, but to our knowledge, the association of HUV and non-Hodgkin lymphoma has not been described so far. A patient with HUV who developed 10 years later a diffuse large B cell lymphoma is reported here.     

Kabuki make-up syndrome (Niikawa-Kuroki syndrome) associated with congenital heart disease.

Kabuki make-up syndrome has been reported mainly among Japanese, so far occurring in more than 20 cases. Among these, however, only one case associated with congenital heart defect has been reported. We have treated three patients with this syndrome and of these two had congenital heart disease. We suggest the possibility that the association of congenital heart disease with Kabuki make-up syndrome may not be fortuitous.     

Congenital heart disease associated with sporadic Kallmann syndrome

A 17-year-old boy with Kallmann syndrome had complex congenital heart disease that included double-outlet right ventricle, d-malposition of the great arteries, right aortic arch, and hypoplastic main pulmonary artery. He had neurosensory hearing loss and mental retardation. The 7 previously reported patients with Kallmann syndrome and cardiac abnormalities were short with height ≥2 standard deviations below the mean for age (5/7), lacked a family history of Kallmann syndrome (6/6), and were mentally retarded (4/4). Patients presenting with Kallmann syndrome and congenital heart defects appear to represent a distinct subgroup of patients with Kallmann syndrome. The cause of this association is unclear, but may involve either autosomal recessive inheritance, sporadic dominant mutation, or a shared teratogenic event during the first trimester of gestation. © 1993 Wiley-Liss, Inc.     

Implications of third heart sounds in patients with valvular heart disease. The Veterans Affairs Cooperative Study on Valvular Heart Disease.

BACKGROUND. The presence of third heart sounds in patients with valvular heart disease is often regarded as a sign of heart failure, but it may also depend on the type of valvular disease. METHODS. We assessed the prevalence of third heart sounds and the relation between third heart sounds and cardiac function in 1281 patients with six types of valvular heart disease. RESULTS. The prevalence of third heart sounds was higher in patients with mitral regurgitation (46 percent) or aortic regurgitation (28 percent) than in those with aortic stenosis (11 percent) or mitral stenosis (8 percent). The left ventricular ejection fraction was significantly lower (P less than 0.001) when a third heart sound was detected in patients with aortic stenosis (0.38, vs. 0.56 in those without third heart sounds) or mixed aortic valve disease (0.40 vs. 0.55). However, the ejection fraction was only slightly lower in patients with mitral regurgitation and third heart sounds (0.51 vs. 0.57, P = 0.03). The pulmonary-capillary wedge pressure was higher (P less than 0.001) when a third heart sound was detected in patients with aortic stenosis (18.6 mm Hg, vs. 12.1 mm Hg in those without third heart sounds). There was no association between the wedge pressure and third heart sounds in patients with mitral regurgitation. The prevalence of third heart sounds increased with the severity of mitral regurgitation. CONCLUSIONS. In patients with mitral regurgitation, third heart sounds are common but do not necessarily reflect left ventricular systolic dysfunction or increased filling pressure. In patients with aortic stenosis, third heart sounds are uncommon but usually indicate the presence of systolic dysfunction and elevated filling pressure.     

Polyangiitis overlap syndrome: cutaneous leukocytoclastic vasculitis associated with polyarteritis nodosa.

A rare case of polyangiitis overlap syndrome is described. The patient was a 25-year-old man who had palpable purpura on his legs which showed leukocytoclastic vasculitis, and polyarteritis nodosa. Superior mesenteric arteriography showed microaneurysms in jejunal branches with focal segmental necrotizing arteritis of small and medium sized muscular arteries in the jejunum. Deposits of IgA and C3 in the superficial blood vessels of the lesional skin were consistent with the features of Henoch-Schönlein purpura. The patient died about two months after initial admission in spite of cytotoxic agent and steroid administration.     

A case of vasculitis syndrome associated with epididymitis]

A case of vasculitis syndrome associated with epididymitis is reported. A 56-year-old male presented with the sudden left testicular pain and fever. He came to the department of urology in our hospital, and was treated with antibiotics and anti-tuberculous drugs. However, the testicular pain was not relieved as well as the fever. In January 5, 1993, he was admitted. He had lost 6 kg. Physical examination revealed pyrexia and blood pressure of 150/91 mmHg. Laboratory finding revealed leukocytosis, thrombocytosis, and erythrocyte sedimentation rate of 100 mm/hr. On serological examination, the C-reactive protein was positive. We suspected this case as a polyarteritis nodosa, then he was given prednisolone. On January 20, 1993, left orchidectomy and spermatic cord biopsy was performed and non-necrotizing vasculitis was found. On August 10, cyclophoaphamide was added and his symptom disappeared. Although polyarteritis nodosa is well known as the vasculitis affecting the epididymis, it was rare that initial manifestation was testicular pain. In this case necrotizing vasculitis was not proven, but we diagnosed this case as a vasculitis in which the initial symptom was the epididymitis.     

A case of autoimmune cholangitis associated with Sj?gren's syndrome and arthropathy.

Autoimmune cholangitis (AC) is a recently proposed entity that describes a specific group of patients presenting overlapping features of primary biliary cirrhosis (PBC) and autoimmune hepatitis. The disease is characterized by dinical cholestasis, high titer antinuclear antibody, negative antimitochondrial antibody, and histologically, findings of PBC coexisting with varying degrees of parenchymal inflammation. In this report, we describe a patient with Sjögren''s syndrome who fulfilled the diagnostic criteria of AC associated with unique arthropathy compatible with arthritis of PBC. This case illustrates the unusual coexistence of two diseases that may share similar pathogenic processes.     

Holt-Oram syndrome associated with the hypoplastic left heart syndrome

We present the first case of Holt-Oram syndrome associated with the lethal congenital heart defect of hypoplastic left heart syndrome. The possible pathophysiological link is explored and the need for careful genetic and cardiologic evaluation in these patients is reiterated.     

A syndrome associated with growth depression and amyloid arthropathy in layers: A preliminary report

In heavy breed layers a new syndrome was encountered, associated with impaired growth. The disease developed from 5 to 6 weeks onwards. Morbidity in most flocks was 1 to 4%, but in some cases up to 20%. The affected birds were smaller in size, made a peeping sound like that of younger chickens, and showed a characteristic stiff gait. The stifle and hock joints were swollen. At necropsy 12 out of 66 affected birds (18%) had bronze-coloured livers and 23 out of 66 (35%) had orange-coloured joint deposits. Histology indicated that these birds appeared to have amyloidosis. The amyloid was found to react positively with anti-duck and anti-bovine amyloid protein A antisera on PAP-staining, indicating it to be of the reactive type. The cause of the syndrome is unknown. A possible association with infectious agents (Streptococcus faecalis and reovirus) is discussed.     

Canine leishmaniasis associated with systemic vasculitis in two dogs.

Two cases of canine leishmaniasis associated with systemic necrotizing vasculitis are described. The main macroscopic lesions were haemorrhagic in type; histopathological changes confirmed a vascular lesion affecting small arteries of several organs (skin, intestinal tract, kidney, urinary bladder, mesenteric lymph nodes, adrenal gland, myocardium, lung, eye and choroid plexus). The presence of the parasite was confirmed with a specific immunocytochemical stain. The possibility of an immunological aetiology of the vasculitis and its classification is discussed.