Annular Eruptive Pseudoangiomatosis and Adenovirus Infection: A Novel Clinical Variant of Paraviral Exanthems and a Novel Virus Association.

Eruptive pseudoangiomatosis is a distinct exanthem thought to be caused by viruses. The usual rash configu-ration is erythematous papules and macules. An association with echovirus infection has been reported. We present here one adult and one child with this exanthem, supported by clinical, histopathological, and immunohistochemical findings. Both patients presented with prodromal symptoms, widespread angioma-like macules in annular configuration, blanchable telangiectasia, followed by spontaneous remission in 6-8 weeks. Lesional histopathology of the adult patient revealed dilated dermal blood vessels and lymphohistiocytic infiltrates predominated by CD4+ lymphocytes with a 5:1 ratio of CD4:CD8 lymphocytes. No B cells or CD56+ natural killer cells were found. Serology of both patients revealed evidence of active infections by adenoviruses, and a range of other viruses were excluded. We believe that these 2 patients manifested annular eruptive pseudoangio-matosis, a novel variant of the rash with a probable adenovirus association that has not yet been reported.

Upregulated expression of CD30 protein in sclerosing angiomatoid nodular transformation (SANT): studies of additional 4 cases and analyses of 6 cases previously published cases.

Sclerosing angiomatoid nodular transformation (SANT) of spleen is a benign lesion with a distinct morphological and immunohisochemical characteristics. Only Weinred I et al (Virchow Arch 451: 73-9, 2007) reported 6 cases of SANT expressing CD30, of which positive for EBV by in situ hybridization (EBER). 4 cases of SANT were added to investigate the clinicopathological features and focused on the expression of CD30 and EBER combined with the previously published literature. Histologically, individual angiomatoid nodules were sharply delineated by fibrocollagenous stroma with numerous vascular lumens and surrounded by a different population of spindle and ovoid cells. Angiomatoid nodules of all of the 4 cases heterogeneously expressed CD34, CD8, CD68 and diffusely demonstrated CD31 and CD30, but none were positive for EBER. We added these cases with reviewed literature to emphasize and verify the fact that upregulated expression of CD30 in SANT is quite common, which should be taken into consideration when making differential diagnosis.

Sclerosing angiomatoid nodular transformation of the spleen.

Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is a rare benign lesion of the spleen with unknown etiology. SANT is classically considered to be a female-predominant disease, with most of the patients in the 30- to 60-year age group. Most lesions are found incidentally on imaging. Although SANT has specific imaging findings, the differential diagnosis from other splenic tumors or malignant lesions is very difficult. Histopathologically, these tumors reveal multiple confluent angiomatoid nodules; these nodules are surrounded by concentric collagen fibers exhibiting an inflammatory and myofibroblastic response and are accompanied by numerous erythrocytes and siderophages. The nodules are populated by endothelial cells, phenotypically recapitulating normal splenic vasculature, such as sinusoids, capillaries, and small veins. Nuclear atypia is minimal, mitotic figures are extremely rare, and necrosis is consistently absent. This lesion has a unique immunohistochemical profile characterized by CD34(-)CD31(+)CD8(+) sinusoids, CD34(+)CD31(+)CD8(-) capillaries, and CD34(-)CD31(+)CD8(-) small veins. CD68 is positive in macrophages. Splenectomy is a useful and effective technique for the management of SANT. SANT patients have a good prognosis, with no recurrence after splenectomy. In this review, we discuss the current knowledge of SANT of the spleen and its clinical relevance.

Eruptive pseudoangiomatosis: report of an adult case and unifying hypothesis of the pathogenesis of paediatric and adult cases.

One month after the onset of immunosuppressive treatment with corticosteroids and mycophenolate mofetil for a newly diagnosed pemphigus vulgaris, a 50-year-old female patient developed a new eruption clinically and histomorphologically consistent with eruptive pseudoangiomatosis (EP). Its self-limited course further confirmed this diagnosis. Although initially described as a paediatric eruption, meanwhile more adult cases of EP (30 out of a total of 53 cases identified by a Medline search) are reported in the literature. The review of adult cases of EP disclosed some common clinical and epidemiological characteristics: adult EP cases tend to cluster in the Mediterranean region of Europe, develop during the summer months, sometimes in the form of limited micro-epidemics, affect immunocompromised individuals and have lesions confined to the exposed skin sites. These characteristics, together with the exanthematic nature of the disease in children, point to some vector-transmitted infectious agent as the cause of this probably underdiagnosed disease.

Diffuse dermal angiomatosis: A previously undescribed pattern of immunoglobulin and complement deposits in two cases.

Two cases of diffuse dermal angiomatosis are reported in middle-aged women. This rare disease of unknown origin is characterized by increased dermal angiomatosis and ulceration. The clinical and histologic presentations of the presently reported lesions were typical for this disorder. Endothelial cells exhibited a normal immunophenotype. The perivascular basement membranes showed a distribution of collagen alpha chains typical for blood vessels, but not for lymphatics. Immunohistochemistry revealed other undescribed features. At the site of the clinical lesions, linear and granular deposits of immunoglobulins A and M, and complement were found around the vessels and at the dermal-epidermal junction. The same deposits were also found restricted to the dermal-epidermal junction in the peripheral clinically intact skin. No serological signs of auto-immune disorder were detected in one patient. A monoclonal gammopathy was disclosed in the other patient. A pattern of immunoreactant deposits similar to that disclosed in the two patients was not found in the control specimens, and has not been described so far in other types of vascular hyperplasia and neoplasia. A pathogenic role of these deposits is unsettled and should be further explored.

Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances.

A 28-year-old woman presented with generalised livedo reticularis, dementia, epilepsy, and pyramidal and extrapyramidal signs. Multiple focal infarcts were seen on MRI. Angiography demonstrated widespread cerebromeningeal angiomatosis with multiple small and medium size arterial occlusions. A lifelong personal and family history of mental handicap in the absence of anticardiolipin antibodies suggests Divry-Van Bogaert syndrome, not previously been reported in a female. Similarities to Sneddon's syndrome are discussed.

Angiomatosis of skin with local intravascular immunoglobulin deposits, associated with monoclonal gammopathy. A potential cutaneous marker for B-chronic lymphocytic leukemia. A report of unusual case with immunohistochemical and immunofluorescence correlation and review of the literature.

Reactive cutaneous vascular proliferation or angiomatosis is associated with various conditions, but is rarely seen secondary to vascular occlusion. We report an unusual case of a 79-year-old female who presented with 8 month history of purpuric facial plaques, with painful crusted ulceration of the nose, later developing similar eruptions on hands, thighs and trunk. Biopsies showed marked angioproliferation, with intravascular (IV) hyaline deposits (PAS+, fibrin+/-; IgM+, fibrinogen+, and C3+), associated with endothelial hyperplasia (Factor VIII+, Vimentin+). Immunofluorescence showed IV IgM, fibrinogen, and granular C3 deposits within vessel walls. Initially, extensive investigations only showed minimal monoclonal gammopathy of undetermined significance (MGUS) and repeatedly negative cryoglobulins. After a 3-year follow-up, the patient developed chronic lymphocytic leukemia (B-CLL). This case illustrates a difficult diagnostic challenge. Although this condition resembles other forms of reactive angiomatosis, it shows distinct features and should be considered in the differential diagnosis of unusual vascular proliferations of the skin. The cutaneous lesions are also considered a potential marker for an underlying systemic condition, which may require prolonged clinical follow-up. We believe this condition to be related to other rare cutaneous vascular proliferations associated with plasma cell and lymphoproliferative disorders. Furthermore, we suggest a common pathogenetic pathway resulting from the IV immunoglobulin deposits causing vascular injury, finally leading to the angiomatosis.

Rochalimaea henselae infection. A new zoonosis with the domestic cat as reservoir.

OBJECTIVE: To determine the reservoir and vector(s) for Rochalimaea henselae, a causative agent of bacillary angiomatosis (BA) and cat scratch disease, and to estimate the percentage of domestic cats with R henselae bacteremia in the Greater San Francisco Bay Region of Northern California. DESIGN: Hospital-based survey of patients diagnosed with BA who also had significant exposure to at least one pet cat, as well as a convenience sampling of pet or impounded cats for prevalence of Rochalimaea bacteremia. SETTING: Community and university hospitals and clinics; veterinary clinics treating privately owned or impounded cats. PATIENTS: Patients with or without human immunodeficiency virus infection, with biopsy-confirmed BA, who had prolonged exposure to pet cats prior to developing BA. MAIN OUTCOME MEASURES: Cultures and laboratory studies were performed on blood drawn from pet cats associated with patients with BA. The Rochalimaea species infecting pet cats and fleas and causing the BA lesions in human contacts of these cats was identified by culture, polymerase chain reaction-restriction fragment length polymorphism analysis, and DNA sequencing. The presence of R henselae bacteremia in pet cats was documented, and predictor variables for culture positivity were evaluated. RESULTS: Four patients diagnosed with BA who had prolonged contact with seven pet cats were identified. The Rochalimaea species causing BA lesions in these patients was determined to be R henselae. The seven pet cats were found to be bacteremic with R henselae; this bacterium was also detected in fleas taken from an infected cat by both direct culture and polymerase chain reaction. Blood samples were cultured from pet and impounded cats (N = 61) in the Greater San Francisco Bay Region, and R henselae was isolated from 41% (25/61) of these cats. CONCLUSION: We have documented that the domestic cat serves as a major persistent reservoir for R henselae, with prolonged, asymptomatic bacteremia from which humans, especially the immunocompromised, may acquire potentially serious infections. Antibiotic treatment of infected cats and control of flea infestation are potential strategies for decreasing human exposure to R henselae.

[Hippel-Lindau syndrome: morphology and immunohistochemistry of tumors related to this syndrome]. / Hippel-Lindau-Syndrom: Morphologie und immunhistochemisches Verhalten einiger dem Syndrom zugeordneter Tumoren.

Hippel Lindau syndrome (HLS), inherited as a simple dominant trait, is characterized by angiomatosis in the brain and retina, and also by cysts and tumours in various abdominal organs. Microscopically there is a striking morphological similarity between some of these tumours and especially between those in the brain (hemangioblastomas) and in the kidneys (renal cell carcinomas). Biopsy and autopsy material from two patients with HLS was examined chiefly by immunohistochemical methods, to investigate further the origin of these tumours. The cerebral hemangioblastomas of both patients showed tumour cells with a positive immunohistochemical reaction for neuron-specific enolase (NSE), suggesting a neural or neuroendocrine origin, while corresponding investigation of the kidney tumours did not produce similar clear results. Systemic immunohistochemical investigation of all tumours related to this syndrome is recommended.

Reassessment of malignant "angioendotheliomatosis". Evidence in favor of its reclassification as "intravascular lymphomatosis".

Malignant angioendotheliomatosis (MAE) is a lethal intravascular proliferation which has been thought to be of endothelial origin. In order to characterize its cellular nature, we studied 15 cases of MAE immunocytochemically, using antisera for factor VIII-related antigen, cytokeratin, epithelial membrane antigen, vimentin, blood group isoantigens, thoracic duct lining cell antigens (TDLCA), common leukocyte antigen, and Ulex europaeus I lectin. In 14 of 15 cases, common leukocyte antigen was observed in malignant intravascular cells. Similar reactivity for factor VIII-related antigen was present in 14 cases, but was largely restricted to cells enmeshed in fibrin-platelet thrombi, and probably represents adsorption of platelet-derived factor VIII by tumor cells. All cases failed to bind Ulex europaeus lectin and lacked immunoreactivity for TDLCA, cytokeratin, epithelial membrane antigen, and blood group isoantigens; two manifested positivity for vimentin. Immunofluorescent microscopy of frozen tissue in one case showed monoclonal IgM-kappa immunoglobulin on the surfaces of tumor cells. Electron-microscopic study of three cases disclosed a predominant cell type lacking features of epithelial or endothelial differentiation; a minor cell population displayed endothelial characteristics and was thought to be reactive. Four patients with typical MAE also had extravascular large-cell lymphoma in lymph nodes, spleen, adrenal glands, stomach, or soft tissues. Six patients showed clinical evidence of autoimmune disease. These results suggest that MAE displays lymphoid rather than endothelial differentiation.