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We report the unusual association of normocomplementemic type I membranoproliferative glomerulonephritis in a 10-year-old girl with sparse red hair, absent eyebrows and eyelashes, cutaneous telangiectasias, and an atrial septal defect.
The purpose of this nonrandomized case-review study was to compare the outcome of stripping and CHIVA for treatment of varicose veins in the lower extremities in our department. Outcome was evaluated by independent physicians. A total of 85 patients underwent saphenous vein stripping in association with phlebectomy and 90 patients underwent CHIVA cure. The duration of follow-up was 3 years. Study criteria were (1) presence of varicose veins as a cause of failure (1.1% in the CHIVA group vs. 15.3% in the stripping group), (2) appearance of telangiectasia (8.9% in the CHIVA group vs. 65.9% in the stripping group), (3) patient dissatisfaction rate (3.3% in the CHIVA group vs. 16.5% in the stripping group), (4) postoperative symptoms as a cause of failure (1.1% in the CHIVA group vs. 21.2% in the stripping group), and (5) saphenous nerve injury (1 patient in the CHIVA group vs. 16 in the stripping group). Differences between all five criteria were significantly in favor of the CHIVA group as compared to saphenous vein stripping with phlebectomy. Clinical results at 3 years are better for patients treated with CHIVA than stripping with regard to presence of varicose veins, clinical symptoms, presence of telangiectasia, cosmetic satisfaction, and neurologic complications. Data in our series of CHIVA treatments are comparable to those reported in the literature and better than those described in three series of stripping procedures with 3-year follow-up. A prospective randomized study is now underway to confirm these findings.
We report a case of congenital telangiectatic focal nodular hyperplasia, a rare variant form of the disease. The patient was a 2-month-old boy whose parents noticed abdominal distention about 2 weeks after birth, and ultrasonogram revealed a large mass in the liver. He underwent right lobectomy, and gross findings showed an ill-defined mass without any central scar. Histologic findings demonstrated proliferating hepatocytes without atypia arranged in cords of 1- or 2-cell thickness with marked sinusoidal dilatation and extramedullary hematopoiesis. In addition, a significantly increased Ki-67 labeling index in the tumor compared with non-tumor liver cells, and cytogenetic analysis of 23 G-banded metaphase preparations revealed 3 abnormal karyotypes, suggesting hyperplastic or neoplastic features. To the best of our knowledge, the present case is only the third documented case of congenital telangiectatic focal nodular hyperplasia.
The use of lasers for the treatment of cutaneous vascular disorders has expanded rapidly since its inception in the early 1960s. The flashlamp-pumped pulsed tunable dye laser (PDL), which is the best laser system for the treatment of port-wine stains (PWS), is based on the premise of selective photothermolysis. Recent literature demonstrates enhanced selective photothermolysis by modifying the PDL to include a longer pulse width, a longer wavelength and higher energy fluences through the use of dynamic cooling. Multiple pulse laser irradiation, a new innovative method, shows promising preliminary results in eradicating hypertrophic PWS. In the treatment of capillary haemangiomas, previous uncontrolled studies suggest benefit with early PDL therapy but a recent randomised controlled trial does not support this claim. Mixed haemangiomas respond poorly to PDL treatment, uncomplicated tumours are best left to involute spontaneously whilst life/organ-threatening lesions require early active intervention. The efficacy of lasers in the treatment of telangiectatic leg veins (TLV) remains controversial. The literature revealed many conflicting claims. Larger and properly controlled studies are necessary to better define the role of lasers in the treatment of TLV.
The response of rosacea-associated erythema and telangiectasia to treatment with the pulsed tunable dye laser (PDL) was evaluated in 12 patients. Improvements in erythema, telangiectasia, flushing, the physician's and the patient's perception of overall severity, treatment tolerability, and the adverse side effects were examined. With the sole exception of the patient's assessment of overall severity we have found highly significant improvements in all other parameters evaluated, with excellent tolerance of treatment. We therefore conclude that the PDL is a safe and effective treatment for the erythemato-telangiectatic component of rosacea.
Whilst most facial telangiectasias respond well to short-pulse-duration pulsed dye laser therapy, studies have shown that for the treatment of larger vessels these short-duration pulses are sub-optimal. Long-pulse frequency-doubled neodymium:YAG lasers have been introduced with pulse durations ranging from 1-50 ms and treatment beam diameters of up to 4 mm. We report the results of KTP/532 nm laser treatment for superficial vascular skin lesions. The aim was to determine the efficacy of the KTP/532 nm laser in the treatment of superficial cutaneous vascular lesions at a regional dermatology centre in a 2 year retrospective analysis. Patients were referred from general dermatology clinics to a purpose-built laser facility. A test dose was performed at the initial consultation and thereafter patients were reviewed and treated at 6 week intervals. Outcome was graded into five classifications by the patient and operator independently based on photographic records: clear, marked improvement, partial response, poor response, and no change or worsening. Over the 2 year period, 204 patients with 246 diagnoses were treated [156 female; median age 41 (range 1-74) years; Fitzpatrick skin types I-III]. Equal numbers of spider angioma (102) and facial telangiectasia (102) were treated. Of those patients who completed treatment and follow up, 57/58 (98%) of spider angiomas and 44/49 (90%) of facial telangiectasia markedly improved or cleared. Satisfactory treatment outcomes, with one clearance and two partial responses, occurred in three of five patients with port-wine stain. Few patients experienced adverse effects: two declined further treatment due to pain, and a small area of minimal superficial scarring developed in one case. Two patients developed mild persistent post-inflammatory hyperpigmentation, and one subject experienced an episode of acute facial erythema, swelling and blistering after one treatment. The KTP/532 nm frequency-doubled neodymium:YAG laser is a safe and effective treatment for common superficial cutaneous vascular lesions in patients with Fitzpatrick skin types I-III.
A consistently effective treatment for small leg veins (< or = 1.0 mm) is still being sought. The efficacy of an 810 nm diode laser in vein removal was assessed in a preliminary study. Fifteen females, skin types I to III, vein diameters 0.5-1 mm, aged from 25 to 42 years, participated in the study. An 810 nm diode laser (90 W, 20 ms/pulse, 10 Hz rep rate, 4.0 mm hand piece) was applied along the target veins. Biopsies were taken from two patients before and after the first treatment session. No compression was applied post-treatment. Four weeks later, a second treatment was given. Results were assessed subjectively from the patients' satisfaction index (SI) and objectively from clinical photography done by an independent clinician, who also judged the venous morphology before and 4 weeks after the second session. All patients completed the trial. Pain was moderate to severe at the time of treatment and erythema which was mild, which was seen in all 15 patients; oedema occurred in 12 patients and blistering in only one. No scarring was noticed. The overall satisfaction indices at the 4- and 8-week assessments were 20.7% and 55.1%, respectively. No patient got worse. The objective evaluations at the 4- and 8-week assessments showed increasing improvement in all aspects examined. Pain at the time of treatment was a problem for all patients, so epidermal cooling should be added. Despite this, the 810 laser diode was an interesting and promising device for treatment of small leg veins, warranting further study in larger patient cohorts with a longer-term follow up.
The theory of selective photo-thermolysis (SP) suggests the occurrence of optimal damage when the laser pulse duration (PD) equals the target thermal relaxation time (Tr). This study examines the clinical relevance of this theory. The study design and methods consisted of prospective, randomised, blinded, controlled study of KTP 532-nm laser treatment of 28 telangiectatic facial vessels. The PD varied from 0.13 to 1.71 times Tr (factor of 13). All vessels achieved marked improvement or clearance. The analysis of proportions reaching clearance over marked improvement for small and large calibre vessels shows a better outcome when the PD is nearer to the Tr (86% vs 57%; P = 0.09, 95% CI for difference -3% to 60%). Logistic regression analysis shows that, as the ratio of PD to Tr increases (PD near to or higher than Tr), the chance of clearing improves (P = 0.169; 95% CI 0.34 to 341). This study has shown that, for the range of vessels investigated: (1) marked improvement or clearance may be achieved using PDs that range between 0.13 and 1.71 times the Tr; and (2) it also reveals an overall trend towards a higher proportion of clearance when the PD is near to or higher than the Tr.
We report the results of endoscopic laser therapy on 59 patients with upper gastrointestinal vascular ectasia. After 300 sessions, complications were two nonbleeding and three bleeding ulcers, treated successfully with endoscopic therapy. After treatment, blood transfusions were abolished in 61% and reduced in 22% of the patients, whereas 17% of the patients were nonresponders and 5% died for bleeding. Treatment outcome correlated with endoscopic healing, number of yearly transfusions, and lesions' localization. Patients, who did not respond to laser therapy by the sixth session, showed no improvement afterwards. Four patients with persistence of bleeding despite laser therapy underwent surgery and in three of them, a long-term control of bleeding was obtained. In conclusion, laser therapy was safe and effective; nonetheless, surgical treatment should be considered, after adequate staging, for those patients receiving more than 10 blood units per year or who have undergone more than six laser sessions without improvement.
BACKGROUND: Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The skin is the most frequently affected organ. Cutaneous mastocytosis, including urticaria pigmentosa, solitary mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans (TMEP), is usually distinguished from systemic mastocytosis. TMEP is characterized mainly by telangiectatic macules. OBJECTIVE AND METHODS: We report a case of TMEP with an unusual clinical presentation as a solitary plaque of telangiectatic macules. A renal clear cell carcinoma was detected in a workup for systemic mastocytosis. We discuss the clinical and histological findings and treatment of TMEP. CONCLUSIONS: TMEP is a rare form of mastocytosis, which occurs mainly in adults, generally has a good prognosis, and little tendency to urticate or show constitutional symptoms. Clinicians should consider this disorder when confronted with a progressive atypical telangiectatic lesion. However, the malignant neoplasm also found in this patient is of uncertain significance.
BACKGROUND: Intravascular lymphoma is a rare and aggressive disease with abnormal lymphocytes confined within blood vessels. Cutaneous findings are common in the presentation of this disease. OBJECTIVES: The objectives of this article are (1) to describe a case of intravascular lymphoma treated with chemotherapy and rituximab and (2) to review the literature with respect to skin findings and outcomes of these patients after chemotherapy. PATIENT: Our patient had an unusual presentation of intravascular lymphoma characterized by tender, slightly indurated plaques composed of dense telangectatic vessels. RESULTS: Our patient responded to combination chemotherapy with the addition of a partial course of rituximab (anti-CD20 monoclonal antibody). CONCLUSION: Intravascular lymphoma commonly presents with skin findings of tender indurated plaques and nodules. Although a number of clinical variations exist, telangectatic plaques are rarely described. Including our case, 50 patients have been reported after treatment with chemotherapy. Approximately 46% of treated patients exhibit either partial or complete response to chemotherapy. This is the first reported case of intravascular lymphoma treated successfully with the addition of rituximab. Overall, this malignancy remains an unusual and aggressive disease. Recognition of the cutaneous findings and early treatment with chemotherapy may alter the course of this disease. Rituximab may be an important addition to combination chemotherapy in treating intravascular lymphoma.
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BACKGROUND AND PURPOSE: Radiotherapy after breast-conserving surgery is commonly applied to reduce recurrence of breast cancer but may cause acute and late side effects. To identify prognostic factors for the development of late toxicity after radiotherapy, we conducted a prospective study of breast cancer patients. PATIENTS AND METHODS: We assessed late complications of radiotherapy and collected information on epidemiologic factors in a cohort of breast cancer patients who had received radiotherapy after breast-conserving surgery. Among 416 patients with complete follow-up data, the association between possible risk factors and development of late complications was evaluated using multivariate logistic regression analysis. RESULTS: After a median follow-up time of 51 months, 131 (31.4%) patients presented with telangiectasia and 28 (6.7%) patients with fibrosis. We observed a strong association between development of telangiectasia and fibrosis (p < 0.01). Increasing age of the patient was a risk factor for both telangiectasia and fibrosis (p-value for trend <0.01 and 0.03, respectively). Patients with acute skin toxicity (odds ratio (OR) 1.8, 95% confidence interval (CI) 1.0-3.1) were at higher risk to develop telangiectasia. Long-term smoking was associated with a significant increase in risk of telangiectasia compared to non-smokers (OR 2.3, 95% CI 1.2-4.6). CONCLUSIONS: Our study revealed several factors other than radiation dose that may predispose to late complications following radiotherapy. Further understanding of differences in response to irradiation may advance individualized treatment and improve cosmetic outcome.
Ten patients with pulmonary hypertension associated with diffuse systemic sclerosis (1 patient), the CREST syndrome (calcinosis cutis, Reynaud's phenomenon, esophageal dysmotility, sclerodactyl, telangiectasia) (6 patients) and mixed connective tissue disease (3 patients) were studied to assess the effect of oral nifedipine on pulmonary and systemic hemodynamics. Each patient underwent right-sided cardiac catheterization just before nifedipine administration. Thereafter, oral nifedipine was administered in 10 mg increments every 90 minutes until pulmonary vascular resistance normalized or a total dose of 30 mg was achieved. Hemodynamic measurements were obtained at 30-minute intervals for 3 hours, then hourly for 9 hours (acute study). Hemodynamic studies were repeated 3 to 6 months after the initial catheterization with the minimum dose of oral nifedipine (administered every 8 hours) required to achieve maximal reduction of pulmonary vascular resistance in the acute study (long-term study). In the acute study, oral nifedipine produced a significant decrease in mean pulmonary vascular resistance from 6.3 +/- 3.8 to 4.3 +/- 3.6 U (p less than 0.001). Similar changes in pulmonary vascular resistance were noted in the long-term study (n = 6). The results indicate that oral nifedipine is capable of producing an acute and sustained reduction in pulmonary vascular resistance in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease.
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A patient with acquired von Willebrand disease and gastrointestinal telangiectasia is described. He presented with the recent onset of spontaneous hemorrhage and demonstrated a prolonged bleeding time, reduced factor VIII coagulant (FVIII:C), and undetectable factor VIII-related antigen (FVIIIR:AG) and ristocetin cofactor (FVIIIR:WF). Following transfusion of cryoprecipitate, there was a smaller than expected immediate increase in FVIII:C, FVIIIR:WF and FVIIIR:AG, with a rapid return to baseline levels and no secondary increase in FVIII:C. An inhibitor could be demonstrated in the patient's plasma which markedly decreased the level of FVIIIR:AG in normal plasma whereas it only weakly decreased the activity of FVIIIR:WF and FVIII:C. The inhibitor was contained in the immunoglobulin G(IgG) fraction of plasma and lacked precipitating properties. This inhibitor demonstrates a specificity not previously seen in spontaneous antifactor VIII antibodies. Our report also demonstrates that ristocetin-induced agglutination of platelets is not always the most sensitive method for detecting an inhibitor in acquired von Willebrand disease.
The CREST syndrome is a variant of systemic sclerosis characterized by the presence of calcinosis. Raynaud's phenomenon, esophageal motility abnormalities, sclerodactyly and telangiectasia. The serums of 27 patients with the CREST syndrome have been examined for the presence of antinuclear antibodies. Twenty-six of 27 (98 percent) serums contained high titers (> one:80) of an antibody that produces a discrete speckled pattern of immunofluorescence on a human laryngeal carcinoma cell line (HEp-2). The antibody has been shown to react with the centromeric region of metaphase chromosomes. This antibody was also found in three of 14 patients with Raynaud's disease, in one of 60 patients with systemic lupus erythematosus, in three of 26 patients with systemic sclerosis with diffuse scleroderma and in one of 15 patients with mixed connective tissue disease. The antibody was not detected in the serums of patients with rheumatoid arthritis. Sjögren's sicca complex or linear scleroderma. Patients with osteoarthritis who were age- and sex-matched to the group with the CREST syndrome did not have anticentromere antibodies. Autoantibodies found in other connective tissue diseases (anti-DNA, anti-RNP, Sjögren's syndrome antigen B (anti-SS-B) were not found in serums from patients with the CREST syndrome. A case report illustrating the appearance of the anticentromere antibody at a time when Raynaud's phenomenon antedated the clinical diagnosis of CREST syndrome is presented.
A case of gastrointestinal bleeding in a 73-year-old man secondary to vascular ectasia is presented. The use of the 99m technetium-tagged erythrocyte scanning to localize the bleeding site and arteriography to document a vascular malformation strongly suggested this diagnosis. Elective hemicolectomy failed to eliminate bleeding, however. Endoscopic and surgical therapies are available for this condition, but the relative frequency of postoperative rebleeding requires conservatism in recommending surgical excision.