Local angioedema following sun exposures: a report of five cases.

Angioedema is a hereditary or acquired disease characterized by localized non-pitting swelling of the subcutaneous tissue which can affect either skin or mucous membranes. Acquired angioedema can often be related to a heterogeneous group of etiological factors including physical stimuli, although up to 38% of cases remain idiopathic. We describe 5 patients who developed an angioedema following sun exposures. All patients reported an intensely stinging angioedema strictly limited to face and extremities, when exposed to solar light. Urticarial wheals were never observed or reported by patients, and oral antihistamines proved to be of no help in preventing or improving the condition of lesions. Laboratory and phototesting data allowed ruling out all other acquired or inherited diseases characterized by photosensitivity. We propose that solar angioedema should be considered a novel clinical entity.

Renal involvement in Churg-Strauss syndrome.

BACKGROUND: Churg-Strauss syndrome (CSS) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected patients. METHODS: We examined the prevalence, clinicopathologic features, and prognosis of renal disease in 116 patients with CSS. RESULTS: There were 48 men and 68 women with a mean age of 51.9 years (range, 18 to 86 years). Signs of renal abnormalities were present in 31 patients (26.7%). Rapidly progressive renal insufficiency was documented in 16 patients (13.8%); urinary abnormalities, 14 patients (12.1%); and chronic renal impairment, 1 patient. There were 3 additional cases of obstructive uropathy. Sixteen patients underwent renal biopsy, which showed necrotizing crescentic glomerulonephritis in 11 patients. Other diagnoses were eosinophilic interstitial nephritis, mesangial glomerulonephritis, and focal sclerosis. Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 patients (75.0%) with nephropathy versus 19 of 74 patients without (25.7%; P < 0.001). In particular, all patients with necrotizing crescentic glomerulonephritis were ANCA positive. After a median follow-up of 4.5 years, 10 patients died (5 patients with nephropathy) and 7 patients developed mild chronic renal insufficiency. Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in patients with nephropathy and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10). CONCLUSION: Renal abnormalities are present in about one quarter of patients with CSS. The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good.

Cinnarizine is a useful and well-tolerated drug in the treatment of acquired cold urticaria (ACU).

Old generation H1-type antihistamines are the standard therapeutic option for acquired cold urticaria (ACU), but adverse effects are common. New antihistamines are well tolerated but efficacy is often poor. The present study aims to evaluate efficacy and safety of cinnarizine in the treatment of ACU patients intolerant to old antihistamines and resistant to new drugs. We studied 14 patients (4 males and 10 females). Mean duration of the disease was 48.9 (range 7-102) months. Cold cube test was positive in 78.6 % of patients. Cold urticaria was idiopathic in 10 (71.4 %) patients. Cryoglobulins were detected in the serum of 4 cases (28.6 %). Cinnarizine (25 mg t.i.d.) was administered for 3 months, and then it was gently tapered off and stopped within 2 months. A complete or good response was obtained in 8 (57.1 %) and 2 (14.3 %) patients, respectively. Only two patients were unresponsive (21.4 %). Tapering off or stopping cinnarizine was followed by the relapse of cold urticaria in 7 cases (50.0 %). These patients were amenable to a second treatment cycle. Six patients (42.9 %) had a persistent remission. A patient interrupted the therapy because of severe vertigo. Three patients reported mild and transitory adverse effects including epigastralgia, weight gain and drowsiness. In conclusion, cinnarizine at high doses may be considered as an effective and well-tolerated treatment for ACU.

Chronic cough and eosinophilic esophagitis: an uncommon association.

An increasing number of children, usually with gastrointestinal symptoms, is diagnosed with eosinophilic esophagitis (EE), and a particular subset of these patients complains of airway manifestations. We present the case of a 2-year-old child with chronic dry cough in whom EE was found after a first diagnosis of gastroesophageal reflux disease (GERD) due to pathological 24-hour esophageal pH monitoring. Traditional allergologic tests were negative, while patch tests were diagnostic for cow's milk allergy. We discuss the intriguing relationship between GERD and EE and the use of patch test for the allergologic screening of patients.

High prevalence of atopy, but not of asthma, among children in an industrialized area in North Italy: the role of familial and environmental factors--a population-based study.

The prevalence of atopy and asthma, and their association with familial and environmental factors were investigated among 13- to 14-yr-old children living in Brescia, an industrialized town in North Italy. All the 1450 children attending primary school in the town were invited to participate, and 967 of them (66.7%, 493 males) provided a valid questionnaire filled in by their parents at home. We used a modified version of the questionnaire adopted in the Italian Study of Respiratory Disorders in Childhood and Environment, which is an extended version of the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. Six hundred and twenty-eight subjects underwent skin prick test (SPT), and 308 of them (49%) were positive for at least one of the 12 allergen extracts commonly employed. Ninety-nine children (10.2%) had a physician's diagnosis of asthma - 12.4% of the males and 8.0% of the females (p = 0.03). The prevalence of wheezing in the past 12 months was 6.2%. Atopy was found in 76.8% of the subjects with, and in 45.6% of those without physician's diagnosis of asthma (p < 0.001). Analysis by multiple logistic regression showed an inverse association between physician-diagnosed asthma and female sex (odds ratio, OR = 0.5); presence of relatives in the bedroom in initial years of life (OR = 0.6); attending day care (OR = 0.4) and infant school (OR = 0.4); a positive association with parental history of wheezing (OR = 2.5) and asthma (OR = 3.8); and the child's history of asthmatic bronchitis (OR = 31.9) and atopic eczema (OR = 3.8) in the first 2 yr of life. The strength of the associations did not change when restricting the analysis to atopic asthma. In conclusion, atopy and clinical asthma among 13- to 14-yr-old adolescents are significantly associated with some familial and environmental factors, providing further support for the hygiene hypothesis. Prevalence of atopy, but not of asthma, is high in this industrialized area. The strong association found between atopy and clinical asthma suggests that atopy may play a role in causing asthma in genetically predisposed children only.

Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.

OBJECTIVE: Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features. METHODS: Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status. RESULTS: ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P = 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P = 0.019) and heart disease (5.7% versus 22.4%; P = 0.042). CONCLUSION: ANCAs are present in approximately 40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis.