Cytomegalovirus infection is known to cause symptomatic disease in immunocompromised patients, while an infection in immunocompetent individuals normally causes few or no symptoms. We present the case of an im...
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Πέμπτη 18 Ιανουαρίου 2018
Relevant pericardial effusion caused by cytomegalovirus infection in an immunocompetent patient: a case report
Clinical and molecular implications of structural changes to desmosomes and corneodesmosomes
Abstract
Desmosomes provide the main intercellular adhesive properties between epidermal keratinocytes. Their distribution becomes uneven in severe dermatitis, multiple allergies and metabolic wasting syndrome due to desmoglein 1 deficiency and the loss of intercellular adhesion or acantholysis. When keratinocytes differentiate from granular cells into cornified cells, desmosomes are transformed into corneodesmosomes and can provide stronger intercellular adhesion. Degradation of corneodesmosomes is a tightly regulated process involving a number of proteases and their inhibitors. Peripheral corneodesmosomes are protected from proteolytic degradation by the tight junction-related structures around them, and this construction provides the basis for the normal basket weave-like structure of the stratum corneum. In Netherton syndrome, which is caused by an absence of the protease inhibitor lymphoepithelial Kazal-type-related inhibitor, premature degradation of corneodesmosomes occurs due to the overactivation of proteases involved in corneodesmosome degradation. Inflammatory peeling skin disease is caused by the absence of corneodesmosin, a unique component of corneodesmosomes. In this disease, corneodesmosomes are structurally abnormal, and their adhesiveness is compromised, which leads to intercellular splitting between the stratum corneum and stratum granulosum. The better we understand desmosome and corneodesmosome ultrastructure in normal and diseased skin, the clearer the physiological and pathological mechanisms of epidermal integrity become.
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Possible role of plasmacytoid dendritic cells in pityriasis lichenoides
Summary
Background
Plasmacytoid dendritic cells (pDCs) and their product, type I interferons (IFNs), have been implicated in the pathogenesis of several skin disorders characterized by an interface dermatitis (ID) pattern, such as lichen planus (LP). A type I IFN signature has previously been documented in pityriasis lichenoides (PL). Although pDCs are known to be the main source and most potent producers of local type I IFNs, their role in PL has not been investigated.
Aim
To investigate the role of pDCs in PL.
Methods
In total, 20 cases of PL and 20 comparable cases of LP were immunohistochemically tested for pDC occurrence and type I IFN production using anti-blood-derived dendritic cell antigen-2 (BDCA2; a specific pDC marker) and anti-myxovirus protein A (anti-MxA) antibodies (indirect marker of pDC activity), respectively. MxA is a well-established surrogate marker for local type 1 IFN production. A semiquantitative scoring system was used.
Results
pDCs were present in all 40 cases with no statistically significant difference between the two groups. MxA expression was intense and diffuse in the majority of PL and LP cases.
Conclusions
pDCs constitute a central component of the inflammatory infiltrate in PL, suggesting that PL shares with the other entities that exhibit an ID a common pDC-driven process through type I IFN production, which ultimately leads to the cytotoxic attack.
http://ift.tt/2DkqLHQ
Moyamoya tipping point: fatal bilateral MCA territory infarction following cocaine abuse
Description
An adult Caucasian man with a history of polysubstance abuse was found unresponsive by the nursing staff at his rehabilitation centre and intubated by emergency medical services. On examination, he was stuporous, his eyes did not open to stimulation, his pupils were reactive, localised to pain with bilateral upper extremities and demonstrated triple flexion to noxious stimuli in the bilateral lower extremities. Urine toxicology was positive for cocaine metabolites. A CT scan of the head was performed, which showed possible hypodensities in the bilateral temporal lobes. MRI of the brain was performed to further characterise these hypodensities, and it revealed bilateral middle cerebral artery (MCA) infarction and generalised cerebral oedema (figure 1). CT angiogram of the head at this point was performed, which showed narrowing of both MCAs and possible moyamoya pattern on the left (figure 2). This moyamoya-like vascular change was suggested by narrowing of the left MCA and hypervascular lenticulostriate...
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Role of modified Eloesser flap in the treatment of bronchopleural fistula caused by pulmonary coccidioidomycosis
Description
Coccidioidomycosis is a fungal infection by the Coccidioides genus and is usually caused by inhalation of the spores. Patients with diabetes are more likely to present with severe lung disease, especially cavitary lesions.1 2 We depict the case of a 47-year-old man with uncontrolled type 2 diabetes mellitus who presented with a right pulmonary lung abscess due to coccidioidomycosis and underwent a right thoracotomy with partial right upper lobe resection. Intraoperatively the pleura was found to be thickened, and the upper lobe had multiple adhesions and was perforated, creating a purulent bronchopleural fistula. He developed recurrent right-sided empyema due to the fistula and an attempt at a second thoracotomy was unsuccessful. After placement of three failed endobronchial valves, he ultimately had a tunnelled pleural catheter placed. Two weeks later the patient began draining frank pus from the site and a modified Eloesser flap...
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Vanadium allergy following total knee arthroplasty
Allergic reactions to metals following joint arthroplasty represent a rare and poorly understood phenomenon. Much is still unknown regarding the natural history of this complication, and how it can best be prevented and managed. We present a case of a 68-year-old woman who underwent a left total knee arthroplasty for treatment of osteoarthritis. After an initial uneventful postoperative course, she developed a troublesome erythematous rash both around the incision site and over her trunk. Blood testing revealed no evidence of infection and clinically her prosthesis was functioning well. Skin patch testing revealed positive results for vanadium (+) and palladium (+). Her cutaneous symptoms are currently being managed conservatively and have shown a partial response to topical steroids. Revision surgery remains a long-term treatment option should conservative therapy fail; however, it would require a custom-made prosthesis as no standard tibial component is free from vanadium.
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Megaduodenum in a 59-year-old man: a very late postoperative complication after duodenal atresia
Intestinal malformations are common defects of the newborn, treated in experienced centres. Reports on long-term follow-up and associated complications are scarce, possibly leading to misinterpretation of clinical signs and symptoms in adulthood. To prevent treatment errors, it is important that physicians are aware of long-term complications of intestinal malformations.
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