Angiocentric Lesions of the Head and Neck

Angiocentric lesions of the head and neck encompass a variety of benign and malignant lesions. Not unexpectedly the sequelae of an angiocentric process independent of its benign or malignant nature is one of tissue ischemia with a potential for either breakdown or reparative fibrosis. Therefore, the clinical presentations can be very similar despite a varied pathogenesis. Among the benign reactive infiltrates that will be considered are angiocentric eosinophilic fibrosis, Wegener’s granulomatosis, microscopic polyangiitis and cocaine associated mid line facial destruction. We will discuss other conditions which enter into the differential diagnosis either clinically or histologically including Erdheim Chester disease and mid line facial undermining unrelated to an angiocentric event specifically in the context of trigeminal trophic ulcer and relapsing polychondritis. The two main neoplastic conditions exhibiting angiocentricity are in the context of lymphomatoid granulomatosis and NK/T cell lymphoma; hence these two particular hematologic dyscrasias will be discussed in some detail in this review.     

Polyarteritis nodosa: Regression of arterial aneurysms following immunosuppressive and corticosteroid therapy

Angiographic illustration of aneurysmal involvement of renal, hepatic, and splenic arteries in a 10-year-old boy, led to correct diagnosis of polyarteritis nodosa. A follow-up study one year after cyclophosphamide and prednisone therapy showed near complete resolution of the arterial lesions and a marked improvement of renal blood flow.     

Polyarteritis nodosa in a case of familial Mediterranean fever

We describe a 7-year-old boy with familial Mediterranean fever (FMF) complicated by polyarteritis nodosa (PAN) with distinct angiographic findings. On admission, he had abdominal pain, arthralgia, and severe fibromyalgia. During hospitalization, he displayed maculopapular eruptions, high blood pressure, gastrointestinal bleeding, and persistent constitutional symptoms mimicking a vasculitic process, most probably PAN. Renal angiography showed a perfusion defect compatible with a renal infarction secondary to a vasculitic process. He responded well to pulse methylprednisolone therapy with colchicine. We emphasize the rare association of FMF and PAN and the non-aneurysmal angiographic signs of PAN.     

Tongue necrosis due to vasculitis

We describe a 7-year-old boy with necrosis of the tongue due to a generalized vasculitis, with symptoms and signs consistent with periarteritis nodosa and appearing after an infection with streptococci. Approximately one-third of the tongue had to be extirpated.     

T cell receptor (TCR) V gene usage in patients with systemic necrotizing vasculitis.

Wegener's granulomatosis (WG) and polyarteritis nodosa (PAN) are systemic necrotizing vasculitides of unknown etiology. These disorders run a fatal course if untreated. T lymphocytes are implicated in the pathogenesis of WG, since they have been found to infiltrate affected organs, and sIL-2R correlates with disease activity. To elucidate further the role of T cells in necrotizing vasculitis, we have used a panel of 12 TCR V-specific MoAbs to investigate the number of cells expressing certain V alpha and V beta gene segments in the CD4+ and CD8+ subsets of altogether 11 patients with WG or PAN. In the group of patients, we found abnormal expansions of T cells using particular TCR V alpha or V beta gene products. These T cell expansions were more numerous, of a dramatically higher magnitude, and frequently more often found in the CD4 subset, compared with T cell expansions identified in healthy individuals. In long-term studies of the T cell expansions for up to 18 months, a heterogeneous pattern was revealed, with no obvious correlation to clinical features such as disease activity or treatment. Studies of TCR V gene usage in this group of patients may help in understanding the pathogenesis of necrotizing vasculitis, and in the identification of unknown antigens, and may open the possibility to a highly selective immunotherapy by targeting disease-mediating T cells.     

Microscopic polyangiitis and polyarteritis nodosa: How and when do they start?

Objective

To describe initial clinical symptoms attributable to microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN).

Methods

We retrospectively reviewed the medical files of 72 patients (mean followup 6.7 years) with biopsy‐proven MPA (n = 36) or PAN (n = 36).

Results

Initial manifestations were similar in both entities except for peripheral neuropathy (P = 0.02) and gastrointestinal tract involvement (P = 0.006), which were significantly more frequent in PAN, and general signs alone in MPA (8%; P = 0.02). The mean time to diagnosis was 9.8 ± 19.4 months; 35% of the patients died and 26% relapsed; significantly more MPA than PAN patients relapsed (P = 0.03). Time to diagnosis ≥90 days was associated with a trend toward more patients relapsing (P = 0.12), but not with an increased risk of mortality.

Conclusion

Initial symptoms of MPA and PAN are usually nonspecific and last for several months before the diagnosis is made. A longer time to diagnosis is associated with a tendency to a higher relapse rate.

     

Successful treatment of Group A β-hemolytic Streptococcus infection-associated juvenile cutaneous polyarteritis nodosa with tonsillectomy

Cutaneous polyarteritis nodosa (cutaneous PAN) is a form of necrotizing vasculitis of small- and medium-sized arteries, primarily involving the skin. In juvenile cases, cutaneous PAN is known to be frequently associated with Group A β-hemolytic Streptococcus (GAS) infections.

We herein describe the first reported juvenile case of GAS-associated recurrent cutaneous PAN successfully improved with tonsillectomy. To avoid the use of steroids and immunosuppressive drugs, especially in juvenile cases, tonsillectomy is a possible treatment for GAS-associated recurrent cutaneous PAN.     

峡部结节性输卵管炎的子宫输卵管造影与病理对照分析

目的：进一步认识峡部结节性输卵管炎（SIN）的子宫输卵管造影（HSG）表现,提高诊断准确率,为此病的初步诊断提供影像学方法。方法：对28例经病理证实的SIN子宫输卵管造影资料进行回顾性分析,并与定理组织进行对照。结果：28条确诊为SIN的输卵管的SHG表现为：（1）15支间质部,峡部纤细,显影断续,逐渐中断;（2）6支输卵管显影断续,边缘毛糙呈“小龛影样”,;（3）4支间质部,峡部增粗呈棒状,远端显影中断;（4）2例子宫角部不规则“息肉样”充盈缺损阴影,输卵管管径不规则,另有1支表现为伴部轻度积水,结论：HSG诊断SIN,简便,有效,无创伤性,可对SIN作出初步诊断。     

Diclofenac, paracetamol, and vidarabine removal during plasma exchange in polyarteritis nodosa patients.

Since plasma exchange (PE) represents a major treatment for patients suffering from systemic diseases, its influence on the kinetics of three drugs was investigated: vidarabine, used in patients with polyarteritis nodosa associated with hepatitis B virus (eight subjects), and diclofenac and paracetamol for investigative purposes (five subjects). This study confirmed that vidarabine is so rapidly deaminated to form hypoxanthine arabinoside (Hx-Ara) that no detectable concentrations were measured. Hx-Ara levels were used to evaluate vidarabine kinetics; 19.5 +/- 14.6 mg of Hx-Ara were removed by one PE during the first week of treatment (15 mg kg-1 d-1, continuous infusion) and 7.8 +/- 10.2 mg were eliminated by one PE during the second week of treatment (7.5 mg kg-1 d-1, continuous infusion). Based on the vidarabine intake per hour and the resulting quantity of Hx-Ara removed per hour, PE recovery was quite important (ca. 30 per cent), during both the first and second weeks of continuous infusion. Data were subject to large interindividual variability. However, these results do not favor vidarabine dosage supplementation in this indication because the duration of PE is less than 8 per cent of a daily administration period. For paracetamol (1 g, single oral dose) and diclofenac (100 mg, single oral dose), the fractions of drug removed during PE effected within 2 h of drug intake, were respectively 5.0 +/- 3.1 per cent and 13.6 +/- 9.5 per cent, while plasmapheretic clearance reached, respectively, 13.0 +/- 10.7 per cent of the systemic clearance for paracetamol and 23.0 +/- 1.0 per cent for diclofenac.     

PAN-Rh6G能量转移荧光猝灭法测定水样中痕量镉(Ⅱ)

目的：根据能量转移荧光猝灭程度,建立一种荧光猝灭法测定环境水样中痕量镉的新方法.方法：在无水乙醇介质中,Cd^2＋与1-（2-吡啶偶氮）-2-萘酚（PAN）形成PAN-Cd^2＋配合物,其吸收光谱与罗丹明6G（Rh6G）的发射光谱有效重叠,发生能量转移,使Rh6G荧光猝灭,从而建立了痕量镉的荧光猝灭测定新方法.结果：在优化实验条件下,在31～800 ng/ml浓度范围内,Rh6G荧光猝灭程度与PCd^2＋呈现良好的线性关系（r=0.9999）.方法的检出限为9.0 ng/ml.结论：方法灵敏、快速、简便,用于环境水样中痕量镉的测定,结果满意.