Three patients with an unusual stenosing lesion affecting the upper respiratory tract are described. The lesion involved the mucosa of the nasal septum and lateral nasal wall in two cases and the mucosa of the sub-glottic region of the larynx in one. The lesion leads to thickening of the sub-mucosal connective tissues by an usual pathological process. It begins as a focal peri-vascular sub-epithelial exudate of eosinophils accompanied by collections of plasma cells and lymphocytes without fibrinoid necrosis. These foci progress to form extensive areas of peri-vascular fibrosis showing a characteristic angiocentric whorling with an 'onion-skin' pattern. This process leads to thickening and matting of the mucosa which becomes adherent to the underlying structures. As fibrosis progresses, the lympho-plasmacytic component regresses but the eosinophils remain. The fibrosis does not resolve and the consequent stenosis requires surgical relief. No other site appears to be affected by this process, but one case was associated with granuloma faciale. The cause is unknown. We have given the term 'eosinophilic angiocentric fibrosis of the upper respiratory tract' to this condition to encompass its cardinal features.
The expression of MUC5AC and MUC5B shares a similar distribution to normal airways in different states of asthma. The distribution is not affected by topical corticosteroid therapy.
Study purposes were to determine the occurrence rate for preoperative breast pain; describe the characteristics of this pain; evaluate for differences in demographic and clinical characteristics; and evaluate for variations in pro- and anti-inflammatory cytokine genes between women who did and did not report pain. Patients (n=398) were recruited prior to surgery and completed self-report questionnaires on a number of pain characteristics. Genotyping was done using a custom genotyping array. Women (28.2%) who reported breast pain were significantly younger (p < 0.001); more likely to be non-white (p= 0.032); reported significantly lower Karnofsky Performance Status scores (p = 0.008); were less likely to be post menopausal (p = 0.012), and had undergone significantly more biopsies (p=0.006). Carriers of the minor allele for a single nucleotide polymorphism (SNP) in interleukin (IL)1-receptor 1 (IL1R1) (rs2110726) were less likely to report breast pain prior to surgery (p = 0.007). Carriers of the minor allele for a SNP in IL13 (rs1295686) were more likely to report breast pain prior to surgery (p= 0.019). Findings suggest that breast pain occurs in over a quarter of women who are about to undergo breast cancer surgery. Based on phenotypic and genotypic characteristics found, inflammatory mechanisms contribute to preoperative breast pain.
Published series on Churg-Strauss syndrome (CSS) come from tertiary referral centres. We retrospectively studied 23 patients with CSS (18 male) seen over 14 years (1982-1995) in a District General Hospital serving a population of 500,000. Mean age of onset of vasculitis was 57, 10-19 years older than in previous series. The commonest clinical features were asthma (22) and eosinophilia > 1.5 x 10(9)/l (21). Systemic vasculitis involving two or more extrapulmonary organs occurred in 22 patients, with specific organ involvement of nervous system (18), joints (13), muscles (13), lungs (11), skin (11), kidneys (11), heart (10), and bowel (7). Various classification systems were applied including the Lanham criteria, which were met in 19 patients; the American College of Rheumatology criteria, met in 14; Churg and Strauss criteria, met in four; and the Chapel Hill Consensus definition, met only in two. ANCA was detected in 10/17 patients where measured. Treatment included corticosteroids (21), cyclophosphamide (8), azathioprine (9), immunoglobulin (2), and methotrexate (1). During follow-up six patients died, two due to myocardial vasculitis (mean age 52 years), three due to infection (mean age 80 years), and one cause unknown. Significant long-term disability was due to asthma in five and neuropathy in six.
Samples of skin and underlying cord obtained at dermofasciectomy for Dupuytren's contracture have been examined for the presence of smooth muscle alpha-actin (SM alpha-actin), a marker for myofibroblasts. 15 of the 20 samples stained positively for SM alpha-actin corresponding with areas of hypercellular Dupuytren's tissue. In 12 of these 15 samples SM alpha-actin-positive hypercellular Dupuytren's tissue extended into the dermis, in three cases reaching the epidermis. In eight samples, diffusely distributed cells positive for SM alpha-actin and resembling fibroblasts were seen in the dermis. These cells appeared to be separate from the Dupuytren's foci. The presence of hypercellular foci and isolated fibroblasts positive for SM alpha-actin within the dermis may explain the high recurrence rate of Dupuytren's disease after fasciectomy.
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