A case of necrotizing fasciitis with septic shock in a cat caused by Acinetobacter baumannii

A 4-year-old, neutered female, domestic shorthair cat admitted to the animal hospital for recurrent constipation presumed to be due to post-traumatic injuries, went into shock with signs including fever and ataxia followed by stupor. On the fifth day of hospitalization, the cat developed severe, diffuse oedema of the ventral abdomen with multifocal to coalescing erythematous areas and small vesicle formation. The results of bacteriological cultures of liver, spleen and kidney specimens led to the diagnosis of Acinetobacter baumannii sepsis. Histopathological findings of skin samples taken during necropsy showed an extensive epidermal and dermal necrosis with septic vasculitis and numerous intralesional gram-negative bacteria. Detection of the bla(OXA-51-like) gene specific for A. baumannii by PCR, performed retrospectively on samples of the deep layers of the skin, confirmed the presence of A. baumannii also in the cutaneous lesions. To our knowledge this is the first report of a necrotizing fasciitis with septic shock in a cat caused by A. baumannii.

Wegener's granulomatosis presenting as pyoderma gangrenosum

Half of the patients with Wegener's granulomatosis develop skin lesions due to the systemic vasculitis. Wegener's granulomatosis should be included in the differential diagnostic considerations for necrotic ulcers, including leg ulcers. We present a case which demonstrates the importance of histological evaluation of a skin biopsy from the margin of the ulcer for establishing the diagnosis. Antineutrophil cytoplasmic antibodies with antigen specificity for proteinase 3 (PR3-ANCA) were detected supporting the diagnosis of Wegener granulomatosis. Further evaluation showed involvement of the eyes and kidneys. The ulcer rapidly healed under treatment with cyclophosphamide and corticosteroids.

Experimental pneumococcal meningitis causes central nervous system pathology without inducing the 72-kd heat shock protein

We examined whether experimental pneumococcal meningitis induced the 72-kd heat shock protein (HSP72), a sensitive marker of neuronal stress in other models of central nervous system (CNS) injury. Brain injury was characterized by vasculitis, cerebritis, and abscess formation in the cortex of infected animals. The extent of these changes correlated with the size of the inoculum (P less than 0.003) and with pathophysiologic parameters of disease severity, i.e., cerebrospinal fluid (CSF) lactate (r = 0.61, P less than 0.0001) and CSF glucose concentrations (r = -0.55, P less than 0.0001). Despite the presence of numerous cortical regions having morphologic evidence of injury, HSP72 was not detected in most animals. When present, only rare neurons were HSP72 positive. Western blot analysis of brain samples confirmed the paucity of HSP72 induction. The lack of neuronal HSP72 expression in this model suggests that at least some of the events leading to neuronal injury in meningitis are unique, when compared with CNS diseases associated with HSP72 induction.

[Childhood Henoch-Schönlein syndrome--common and uncommon features, complications, Finkelstein-Seidlmayer variant and management]

Although Henoch-Schönlein syndrome can occur at any age, it is overwhelmingly a disease of childhood. Indeed, Henoch-Schönlein syndrome is the most common vasculitis that affects children. The clinical features of this vasculitis are well documented, and the diagnosis is generally not difficult. This article briefly reviews both common and uncommon clinical aspects of the condition and information concerning therapy. A further focus of this review is recent information concerning abnormalities of immunoglobulin IgA1 glycosylation and the role of aberrantly glycosylated immunoglobulins in the development of Henoch-Schönlein syndrome. The final focus of the article is acute hemorrhagic edema, a benign vasculitis limited to the skin, which is characterized by circinate, medallion-like purpura, and ecchymoses and occurs in children younger than 4 years of age. The nosologic position of acute hemorrhagic edema, which has also been called Finkelstein-Seidlmayer syndrome, as a variant of Henoch-Schönlein syndrome is the subject of considerable debate, but most authors agree that there are sufficient clinical and prognostic differences to consider it a separate entity.

Microsurgical repair of the sural nerve after nerve biopsy to avoid associated sensory morbidity: a preliminary report

OBJECTIVE: The purpose of this article is to report our preliminary results regarding microsurgical repair of the sural nerve after nerve biopsy, in an attempt to reduce the well-described sensory morbidity and neuroma formation. METHODS: Three patients with a suspected diagnosis of peripheral neuropathy underwent sural nerve biopsies to establish definitive diagnoses. A 10-mm segment of the sural nerve was resected with local anesthesia. After harvesting of the specimen, the proximal and distal nerve stumps were carefully mobilized and united with epineural suture techniques, under a surgical microscope. Sensory evaluations (assessing the presence of hypesthesia/dysesthesia or pain) of the lateral aspect of the foot, in regions designated Areas 1, 2, and 3, were performed before and 6 and 12 months after the biopsies. A visual analog scale was used for pain estimation. RESULTS: The biopsy material was sufficient for histopathological examinations in all cases, leading to conclusive diagnoses (vasculitis in two cases and amyloidosis in one case). The early post-biopsy hypesthesia, which was present for 4 to 8 weeks, improved to preoperative levels as early as 6 months after the nerve repair. Sensory evaluations performed at 6- and 12-month follow-up times demonstrated that none of the patients complained of pain at the biopsy site or distally in the area innervated by the sural nerve. Ultrasonography performed at the 12-month follow-up examination revealed normal sural nerve morphological features, with no neuroma formation, comparable to findings for the contralateral site. CONCLUSION: Microsurgical repair of the sural nerve after biopsy can eliminate or reduce sensory disturbances such as paraesthesia, hypesthesia, and dysesthesia distal to the biopsy site, in the distribution of the sensory innervation of the sural nerve, and can prevent painful neuroma formation. To our knowledge, this article is the first in the literature to report on microsurgical repair of the sural nerve after nerve biopsy. Decreased side effects suggest that this technique can become a standard procedure after sural nerve biopsy, which is commonly required to establish the diagnosis of various diseases, such as peripheral nerve pathological conditions, vasculitis, and amyloidosis. More cases should be analyzed, however, to explore the usefulness of the technique and the reliability of sural nerve biopsy samples in attempts to obtain conclusive diagnoses.

Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature

In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schönlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features.

Fluktuierende Kopfschmerzen und Wesensveränderung bei einem 63-jährigen Patienten

We present a 63 year old man with new onset of fluctuating headache and behavioural changes showing marked inhibition and disorientation. After non invasive and invasive diagnostics an isolated cerebral vasculitis was found. Key results have been found in cerebral MRI and cerebral digital subtraction angiography with irregularities of vessel calibre of the intracerebral arteries. During treatment with high-dose corticosteroid therapy and Cyclophosphamid pulse therapy qualitative disorders and headache rapidly regressed. We discuss differential diagnosis of secondary headache, etiology of cerebral vasculitides, diagnostic challenge and therapy in isolated cerebral vasculitis.

State of the art: Reproduction and pregnancy in rheumatic diseases

Throughout the last decade, increasing awareness has been raised on issues related to reproduction in rheumatic diseases including basic research to clarify the important role of estrogens in the etiology and pathophysiology of immune/inflammatory diseases. Sub- or infertility is a heterogeneous condition that can be related to immunological mechanisms, to pregnancy loss, to disease burden, to therapy, and to choices in regard to family size. Progress in reproductive medicine has made it possible for more patients with rheumatic disease to have children. Active disease in women with rheumatoid arthritis (RA) affects their children's birth weight and may have long-term effects on their future health status. Pregnancy complications as preeclampsia and intrauterine growth restriction are still increased in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), however, biomarkers can monitor adverse events, and several new therapies may improve outcomes. Pregnancies in women with APS remain a challenge, and better therapies for the obstetric APS are needed. New prospective studies indicate improved outcomes for pregnancies in women with rare diseases like systemic sclerosis and vasculitis. TNF inhibitors hold promise for maintaining remission in rheumatological patients and may be continued at least in the first half of pregnancy. Pre-conceptional counseling and interdisciplinary management of pregnancies are essential for ensuring optimal pregnancy outcomes.

IgA-Nephropathie und Purpura Schönlein-Henoch - ein breites Spektrum

IgA nephropathy is the most common glomerulonephritis in Europe. The disease has been discovered in 1968 in Paris by Jean Berger at the Necker-Children's Hospital. Diagnosis is made by kidney biopsy and requires the presence of mesangial deposits of IgA. This form of glomerulonephritis can be seen in children and adults. In childhood, it most frequently presents within the context of Schoenlein-Henoch purpura. In adulthood, the most common form is limited to the kidney. Schoenlein-Henoch purpura can be seen in adults and manifests as a very aggressive vasculitis, usually in the context of a specific drug intake. The underlying pathophysiological concept today is an insufficient glycosylation of the IgA1 hinge region triggering the formation of autoantibodies against this site. Therapeutic options for the disease are limited. Important is optimal blood pressure control. Selected patients will profit from steroid therapy.

Anaplasma phagocytophilum DNA amplified from lesional skin of seropositive dogs.

Canine granulocytic anaplasmosis (CGA) is caused by the rickettsial microorganism Anaplasma phagocytophilum. CGA is typically characterized by fever, thrombocytopenia, lethargy, anorexia, arthropy, and other nonspecific clinical signs. Skin lesions have been described in naturally infected lambs and humans. The pathophysiology of CGA is not entirely clear, and the persistence of the organism after the resolution of clinical signs has been described. The aim of the study was to investigate if A. phagocytophilum can be detected in canine lesional skin biopsies from A. phagocytophilum-seropositive dogs with etiologically unclear skin lesions that improved after the treatment with doxycycline. Paraffin-embedded lesional skin biopsies were allocated into separate groups: biopsies from A. phagocytophilum-seropositive dogs responsive to treatment with doxycycline (n=12), biopsies from A. phagocytophilum-seronegative dogs (n=2), and biopsies in which skin lesions histopathologically resembled a tick bite (n=10). The serological status of the latter group was unknown. Histology of the seropositive and seronegative dog skin lesions did not indicate an etiology. DNA was extracted, and a conventional PCR for partial 16S rRNA gene was performed. Anaplasma phagocytophilum DNA was amplified from 4/12 seropositive dogs' skin biopsies. All sequences were 100% identical to the prototype A. phagocytophilum human strain (GenBank accession number U02521). Anaplasma phagocytophilum was not amplified from the 2 seronegative and 10 suspected tick bite dogs. Serum antibody titers of the PCR-positive dogs ranged from 1:200 to 1:2048. Histopathologically, a mild-to-moderate perivascular to interstitial dermatitis composed of a mixed cellular infiltrate and mild-to-moderate edema was seen in all seropositive dogs. In 8/12 seropositive dogs, vascular changes as vasculopathy, fibrinoid necrosis of the vessel walls, and leukocytoclastic changes were observed. In summary, our results support the hypothesis that the persistence of A. phagocytophilum in the skin may be causative for otherwise unexplained skin lesions in seropositive dogs.

Systemic Scuticociliatosis (Philasterides dicentrarchi) in sharks.

Scuticociliatosis is an economically important, frequently fatal disease of marine fish in aquaculture, caused by histophagous ciliated protozoa in the subclass Scuticociliatida of the phylum Ciliophora. A rapidly lethal systemic scuticociliate infection is described that affected aquarium-captive zebra sharks (Stegostoma fasciatum), Port Jackson sharks (Heterodontus portusjacksoni), and a Japanese horn shark (Heterodontus japonicus). Animals died unexpectedly or after a brief period of lethargy or behavioral abnormality. Gross findings included necrohemorrhagic hepatitis and increased volumes of celomic fluid. Histologically, 1 or more of a triad of necrotizing hepatitis, necrotizing meningoencephalitis, and thrombosing branchitis were seen in all cases, with necrotizing vasculitis or intravascular fibrinocellular thrombi. Lesions contained variably abundant invading ciliated protozoa. Molecular identification by polymerase chain reaction from formalin-fixed tissues identified these as the scuticociliate Philasterides dicentrarchi (syn. Miamiensis avidus), a novel and potentially emergent pathogen in sharks.

Unusual Eruptions Associated with Mycoplasma pneumoniae Respiratory Infections: Review of the Literature.

BACKGROUND Maculopapular or urticarial eruptions and erythema multiforme sometimes occur in patients affected with Mycoplasma pneumoniae respiratory infections. Further eruptions have also been reported. OBJECTIVE To review the literature addressing M. pneumoniae respiratory infection and rather unusual eruptions. METHODS Computer-based search in the U.S. National Library of Medicine database as well as in the search engine Google. RESULTS We found a possible relationship between M. pneumoniae infection and Fuchs' syndrome (n = 37), varicella-like eruptions (n = 8), Henoch-Schönlein syndrome and further leukocytoclastic vasculitides (n = 21) and erythema nodosum (n = 11). A temporal relationship was also observed with 2 cases of Gianotti-Crosti syndrome. Finally, there exists reasonable evidence that pityriasis rosea Gibert and pityriasis lichenoides et varioliformis acuta Mucha-Habermann are not associated with Mycoplasma infections. CONCLUSION This review implies that M. pneumoniae may cause, in addition to erythematous maculopapular (or urticarial) eruptions and erythema multiforme, Fuchs' syndrome and varicella-like eruptions. Furthermore, there is an intriguing link with leukocytoclastic vasculitides or erythema nodosum that deserves further investigation.

Gallbladder and Pancreas in Henoch-Schönlein Purpura: Review of the Literature

OBJECTIVE Involvement of the pancreato-biliary system has been occasionally noted in Henoch-Schönlein purpura. Furthermore, cases of this vasculitis syndrome sometimes develop in the context of a viral hepatitis or after hepatitis vaccination. METHODS We completed a review of the literature. RESULTS Fifty reports published between 1977 and 2015 were retained for the analysis. A pancreato-biliary involvement was recognized in 34 individually well-described patients (♂:♀ = 19:15) with severe abdominal pain: pancreatitis (N = 20), acalculous cholecystitis (N = 11), both pancreatitis and cholecystitis (N = 3). In all of the pancreatitis patients, full recovery occurred (within ≤3 weeks in three-fourths of the patients). Cholecystectomy was performed in 8 cholecystitis patients. Seventeen Henoch-Schönlein patients (♂:♀ = 9:8) were associated with a viral liver disease and 4 (♂:♀ = 1:3) with a hepatitis vaccination. The vasculitis syndrome rapidly remitted in the 7 patients accompanying hepatitis A or E, in 2 patients of hepatitis B, and in the 4 patients preceded by a vaccination. Henoch-Schönlein purpura seemed to be serious in 5 patients with chronic hepatitis B and in 3 with chronic hepatitis C. CONCLUSIONS This analysis indicates that pancreato-biliary involvement is unusual in Henoch-Schönlein purpura. This complication deserves consideration in patients with especially severe abdominal pain. Finally, viral hepatitides and hepatitis vaccinations seem to be rare triggers of Henoch-Schönlein purpura.

Emergency management of ischemic stroke in children.

OPINION STATEMENT Children who present with acute neurological symptoms suggestive of a stroke need immediate clinical assessment and urgent neuroimaging to confirm diagnosis. Magnetic resonance imaging (MRI) is the investigation of first choice due to limited sensitivity of computed tomography (CT) for detection of ischaemia. Acute monitoring should include monitoring of blood pressure and body temperature, and neurological observations. Surveillance in a paediatric high dependency or intensive care unit and neurosurgical consultation are mandatory in children with large infarcts at risk of developing malignant oedema or haemorrhagic transformation. Thrombolysis and/or endovascular treatment, whilst not currently approved for use in children, may be considered when stroke diagnosis is confirmed within 4.5 to 6 h, provided there are no contraindications on standard adult criteria. Standard treatment consists of aspirin, but anticoagulation therapy is frequently prescribed in stroke due to cardiac disease and extracranial dissection. Steroids and immunosuppression have a definite place in children with proven vasculitis, but their role in focal arteriopathies is less clear. Decompressive craniotomy should be considered in children with deteriorating consciousness or signs of raised intracranial pressure.