Granulomatous vasculitis.

Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Wegener's granulomatosis and Churg-Strauss Syndrome respectively, are systemic granulomatous vasculitides affecting small- and medium-sized blood vessels. Both GPA and EGPA are included within the group of antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides, on the basis of the detection of such autoantibodies in a significant proportion of affected patients. Two main settings of GPA, possibly overlapping each other, are recognized: a localized form, which is limited to the upper airways but is highly relapsing and refractory, and a diffuse form, which is initially more severe but then less commonly recurrent. In EGPA, a prodromic phase characterized by asthma and rhino-sinusitis is followed by an eosinophilic phase, marked by peripheral eosinophilia, and then by a vasculitic phase, in which skin lesions are a prominent feature together with peripheral neuropathy and renal involvement. Polymorphic cutaneous manifestations can occur during the course of both GPA and EGPA, and include palpable purpura, livedo reticularis, papules, nodules, vesiculo-bullae and necrotic-ulcerative lesions most commonly involving the lower extremities; pyoderma gangrenosum-like ulcers and lesions resembling erythema multiforme have been described in GPA and EGPA, respectively. Oral involvement is not uncommon in GPA and may manifest as nonspecific erosive lesions or as a hyperplastic gingivitis named strawberry gingivitis. Considering that skin involvement is common in ANCA-associated vasculitides and may also be their presenting sign, the role of dermatologist is crucial in the early diagnosis of these forms as well as of vasculitis in general.

Eosinophilic granulomatous with polyangiitis complicated by swelling of the oral cavity floor and cervical soft tissue as initial manifestation mimicking IgG4-related disease: A case report.

Eosinophilic granulomatous polyangiitis is a systemic vasculitis associated with bronchial asthma and eosinophilic sinusitis. Here, we describe an unusual presentation of eosinophilic granulomatous polyangiitis that initially manifested as swelling of the oral cavity floor and cervical soft tissue. A 58 year-old Japanese man was diagnosed with bronchial asthma during childhood but did not receive regular medication. Prior to this presentation, he had a persistent cough for over 1 month, and a local physician diagnosed him with bronchial asthma. However, 6 months later, his cough worsened, and a blood test revealed elevated eosinophil levels. Immediately afterward, swelling of the floor of the oral cavity and cervical soft tissue developed. Cellulitis was suspected and antimicrobial treatment was initiated; however, the symptoms persisted and abdominal pain developed. An endoscopic examination revealed duodenitis and a duodenal ulcer. The patient was diagnosed with eosinophilic granulomatous polyangiitis based on three items of the 2022 American College of Rheumatology/European College of Rheumatology classification criteria: obstructive airway disease, blood eosinophil count ≥1 × 109 cells/L, and extravascular eosinophilic infiltration with a score of 10. Oral prednisolone (70 mg/day), intravenous cyclophosphamide (500 mg/m2), and subcutaneous mepolizumab (300 mg every 4 weeks) were administered. The patient's symptoms improved after these treatments, and the eosinophil count and inflammatory marker levels declined. When swelling of the oral cavity floor and cervical soft tissue following an increase in eosinophilia and allergic symptoms occurs, it is crucial to consider the likelihood of eosinophilic granulomatous polyangiitis and collaborate with otolaryngologists and dentists to ensure its prompt identification.

Autoimmune syndrome induced by adjuvants (ASIA) in the Middle East: morphea following silicone implantation.

Morphea and other scleroderma-like skin conditions are occasionally linked with exposure to chemical compounds such as silicone. We treated a 56-year-old woman with generalized severe skin induration accompanied with systemic symptoms and peripheral eosinophilia, which appeared 2.5 years after breast silicone implantation and abdominal liposuction. Blood test results and histopathological examination of her skin suggested the diagnosis of morphea overlapping with eosinophilic fasciitis. Her skin disease was presumed to be an autoimmune reaction to silicone implantation. While the removal of the implants did not improve her illness, treatment with 1 mg/kg prednisone and PUVA bath was initiated, with some improvement. This patient illustrates an example of ASIA (Autoimmune Syndrome Induced by Adjuvants), as her disease appeared following exposure to an adjuvant stimulus, with 'typical', although not well-defined, autoimmune manifestations.

Incidence and prognostic value of eosinophilia in chronic graft-versus-host disease after nonmyeloablative hematopoietic cell transplantation.

Data from a number of cohorts indicate that eosinophilia (Eo) could be associated with better outcomes following allogeneic hematopoietic cell transplant (HCT). However, little is known about its significance and prognostic value in chronic graft-versus-host disease (cGVHD) after nonmyeloablative (NMA) transplantation. Data were collected from 170 patients who underwent HCT using the same preparative regimen and GVHD prophylaxis. Donors were 6/6 HLA-matched siblings and stem cell source was peripheral blood. An eosinophil count of ≥0.5 × 10(9)/L was defined as Eo. Patients were transplanted mainly for lymphoproliferative disorders. Median age and follow-up were 54 years and 58 months, respectively. Incidents of grade II-IV acute GVHD (aGVHD) and cGVHD were 8.2% and 81.2%. Median time from HCT to cGVHD diagnosis was 142 days. Organs involved were: mouth in 80% of patients, skin in 75%, liver in 57%, eyes in 37%, gut in 14%, lungs in 5%, others in 5%. Eo was found in 44% of patients at diagnosis of cGVHD (range: 0.5-4.4 × 10(9)/L). Median time between first appearance of Eo and diagnosis of cGVHD was 4.5 days. We found no correlation between organ involvement and Eo but a lower prevalence of Eo in cGVHD associated with thrombocytopenia (P = .023). Nevertheless, we observed no association among Eo and overall survival (OS), relapse incidence, or nonrelapse mortality (NRM) in the overall cohort, nor in subsets of patients with multiple myeloma and follicular non-Hodgkin lymphoma. Although Eo is observed frequently in cGVHD following NMA transplantation, we report no correlation beween Eo and outcome.

Pruritic rash and eosinophilia in a patient receiving peritoneal dialysis.

A 28-year-old woman with end-stage renal disease maintained on peritoneal dialysis developed a hyperpigmented macular pruritic rash on multiple parts of her body associated with an eosinophilia of 22%. The consulting allergist suspected a silicone allergy from the peritoneal dialysis catheter. A patch test confirmed this diagnosis. Treatment with both topical and systemic steroids was ineffective. Following a living nonrelated renal transplant and removal of the catheter the rash and eosinophilia resolved.

Foreign Body Reaction to Dialysis Chatheter and Peritoneal Fluid Eosinophilia in a Child on Continuous Ambulatory Peritoneal Dialysis.

Foreign body reaction is a tissue response against implanted materials. We described for the first time the eosinophilic peritonitis and foreign body giant cell reaction to dialysis catheter in a nonatopic child on continuous ambulatory peritoneal dialysis. We found tenderness, redness, and swelling without purulent discharge around the peritoneal catheter; increased eosinophil count in cloudy dialysis fluid; and blood and hyperechoic granulomatous formation appearance surrounding the peritoneal catheter on ultrasonography and foreign body giant cell reaction to dialysis catheter in pathologic examination of granulomatous lesionin in our patient. The peritoneal dialysis catheter was removed due to resistance to antibiotic and antihistamine treatments for suspected peritonitis and tunnel infection. Foreign body reaction and eosinophilic peritonitis with eosinophilic cloudy dialysis effluent can exist simultaneously. Foreign body reaction should be considered in the differential diagnosis of exit site and/or tunnel infection. Ultrasonography helps distinguish between foreign body reaction and exit-site or tunnel infection.

Eosinophilic meningitis triggered by implanted Gliadel wafers: case report.

Although carmustine (Gliadel) wafers improve local tumor control and extend the overall survival in patients with malignant glioma, adverse effects have been documented. The authors report the first case of eosinophilic meningitis triggered by the placement of Gliadel wafers. A 61-year-old man with a history of alimentary allergy and glioblastoma in the right frontal lobe underwent resection followed by the implantation of Gliadel wafers. Three weeks later he suffered the sudden onset of headache, vomiting, and progressive consciousness disturbance. Computed tomography revealed enlargement of the ventricular system and subdural space on the side of the tumor. His CSF leukocyte count increased up to 3990 cells/mm3; 95% of the cells were eosinophilic granulocytes (EGs), suggesting eosinophilic meningitis. Laboratory examination showed the patient to have various elevated allergy indicators. The administration of corticosteroids failed to improve his condition. Despite the insertion of a lumbar drain his symptoms failed to improve. He underwent a second surgical intervention to remove the Gliadel wafers. Histologically, EGs had assembled around the wafers. Eosinophilic infiltrate was present in the brain parenchyma around small vessels. After ventriculoperitoneal shunting his course was favorable. A drug lymphocyte stimulation test against the Gliadel wafers failed to demonstrate a positive reaction; polifeprosan, the wafer matrix without 1,3-bis(2-chloroethyl)-1-nitrosourea, yielded a positive reaction. These findings strongly suggest that although extremely rare, polifeprosan (the wafer matrix) can elicit an allergic reaction. When eosinophilic meningitis is suspected after the implantation of Gliadel wafers, their immediate removal should be considered.

Two cases with HSS/DRESS syndrome developing after prosthetic joint surgery: does vancomycin-laden bone cement play a role in this syndrome?

We report two cases of hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (HSS/DRESS) syndrome following systemic and local (via antibiotic laden bone cement (ALBC)) exposures to vancomycin. Both cases developed symptoms 2-4 weeks after the initiation of treatment. They responded to systemic corticosteroid treatment and were cured completely. Various drug groups may cause HSS/DRESS syndrome, and vancomycin-related cases do not exceed 2-5% of the reported cases. Almost all of these cases developed the syndrome following systemic exposure to vancomycin. ALBC seems to be the safer antibiotic administration method, as systemic antibiotic levels did not reach a toxic threshold level. However, local administration may not always be sufficient for bone-related/joint-related infections; these infections may require systemic antibiotics as well. As HSS/DRESS syndrome can mimic infectious diseases, it must be considered during differential diagnosis before suspecting failure of treatment and initiation of a different antibiotic course.

Different clinical picture in early and late onset cortical hyperostosis.

Six cases of Cortical Hyperostosis (C.H.) are presented. The clinical and laboratory features of the disease seem to be related to age at onset. In fact, our cases with a later onset (after the first year of life) are characterized by: a) a longer duration of the disease and a particularly high frequency of relapses; b) the absence of any sign of mandibular involvement; c) the presence of eosinophilia (700-1300/mm3). In two out of three cases having a later onset clinical and laboratory data suggest the hypotesis of a immunoallergic pathogenesis and particularly of cow's milk allergy. A review of the literature appears to confirm the differences between early and late onset forms of C.H. as for as the sites of skeletal involvement and the whole clinical course are concerned.

[Eosinophilic ulcer of the tongue. Pathogenic role of eosinophils]. / Ulcera eosinofílica de la lengua. Papel patogénico del eosinófilo.

A male patient with an ulcerative lesion on the edge of his tongue is present. The histopathologic findings were typical for "Eosinophilic Ulcer of the Tongue". This was contiguous with a carious molar, the remotion of which was followed by total remission of the ulcer. Its possible aetiology and pathogenesis are discussed. Based on the injurious effects of eosinophils mentioned in anothers affections the authors postulate that eosinophils may have an important rol in production or maintainance of this ulcer.

Hyper-IgE syndrome: dental implications.

Hyper-IgE syndrome (HIES) is a rare multisystem disorder with both immunologic and nonimmunological features. It is characterized by extremely elevated IgE serum levels, eczema, and recurrent skin and pulmonary infections. Dental anomalies are often included, such as retention of deciduous teeth together with ectopic eruption or noneruption of permanent teeth. Severe susceptibility to caries and mycotic infections, insufficient transversal diameter of the palate, mucosal plaques, and fissures typically located on the tongue and on the palate are often present. The aim of this study was to review the literature and to report a 6-year observation of 6 patients with HIES (aged 8-39 years) with focus on their oral problems and the treatment provided. The importance of the role of the dentist both in early diagnosis of this syndrome and in monitoring oral conditions was stressed. The dentist can prevent infective complications and intercept the development of malocclusion with a reduction of the need for complex treatment.

Cerebrospinal fluid eosinophilia associated with intraventricular shunts.

CSF eosinophilia (CSF-eo) is uncommon and is usually caused by helminthic infections. However, it has also been found in ∼30% of patients experiencing intraventricular shunt malfunctions. We present a case report and review the conditions associated with CSF-eo and their prophylaxis. An 8 year-old boy with tetraventricular hydrocephalus has had several shunt malfunctions over the last three years. During hospitalization in January 2009 for shunt revision, a transient 30% eosinophilia was detected in his cerebral spinal fluid (CSF) concomitant with Staphylococcus epidermidis infection and long term vancomycin administration. After several shunt replacements and antibiotic treatment, CSF-eo eventually disappeared with good overall clinical response. CSF-eo is a transient and focal event mainly associated with infection, reactions to foreign substances, particles or blood, or obstruction of tubing by normal or fibro-granulomatous tissues. Infection associated with CSF-eo is usually caused by S. epidermidis and Propioniumbacterium acnes. In addition to infection, allergy to silicone and other foreign materials may also be a cause of CSF-eo. We review the diversity of conditions and proposed mechanisms associated with CSF-eo, as well as recommendations for the care of patients with shunts. Detection of CSF-eo has been shown to be a useful indicator of shunt malfunction. As such, it provides physicians with an indicator of a hypersensitivity reaction that is underway or the need to identify bacterial infection. We also highlight the need for improved biocompatibility of shunt hardware and describe strategies to avoid conditions leading to shunt malfunction.