Multiple Telangiectasias Research Articles

Uncommon presentation of a patient with hereditary haemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder. It is clinically characterised by telangiectasia, recurrent epistaxis, and visceral vascular lesions. We report a case of HHT without a significant family history. A 16-year-old girl presented with multiple episodes of bleeding, including uncommon sites, over a period of ten months. She denied a family history of bleeding. Her clinical examination was unremarkable. Investigations including basic and second-line coagulation tests were normal. Subsequently multiple telangiectasias in the right nasal septum and capillary dilatation in the bladder wall were detected. According to Curaçao diagnostic criteria, a diagnosis of HHT was made. As her bleeding was self-limiting, follow up was arranged to monitor complications.

Pulmonary arteriovenous malformations in Rendu-Osler-Weber syndrome.

Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant hereditary disorder. It is characterized by presence of multiple arteriovenous malformations (AVMs) and telangiectasias. This article reports two cases of patients with Rendu-Osler-Weber syndrome who had pulmonary AVMs and underwent successful endovascular treatment. A brief review of the literature shows that up to 50% of patients with the syndrome have pulmonary AVMs and there is usually a positive family history in these patients. These pulmonary AVMs are multiple in 30% of cases and are associated with the most severe disease complications. Most patients are asymptomatic, even in the presence of AVMs with right-left shunts. When these shunts exceed 25% of the total blood volume, dyspnea, cyanosis, digital clubbing, and extracardiac murmurs may occur. Endovascular treatment is safe and offers control of complications from hereditary hemorrhagic telangiectasia and is currently the treatment of choice for these lesions.

СЕМЕЙНЫЙ СЛУЧАЙ НАСЛЕДСТВЕННОЙ ГЕМОРРАГИЧЕСКОЙ ТЕЛЕАНГИЭКТАЗИИ

The article provides an observation of the clinical detection of familial hereditary hemorrhagic 
 telangiectasia (Osler-Weber-Rondu disease), which is manifested by a tumor of the transformation of small 
 foci in multiple telangiectasias, on the skin, mucous and serous membranes, arteriovenous shunts in the internal 
 organs and aneurysms, which is manifested by bleeding. This rare defect had various clinical manifestations 
 in the mother and caused relapses of pulmonary circulation in the child and, as a result, posthemorrhagic ane mia. The given obligatory observation by a pediatrician should be directed to severe symptoms of the disease 
 and obligatory compliance with the requirements and treatment

P437 HEART AND RHEUMATOLOGICAL DISEASE: A PICTURE OF ACUTE HEART FAILURE

Abstract Systemic sclerosis is a connective tissue disease characterized by endothelial dysfunction, hyper reactivity of fibroblasts and immunity abnormality in both humoral and mediated cells. Clinically, in addition to the typical skin involvement, mainly the face and fingers, is frequent involvement of lungs, heart and kidneys. Cardiac involvement, in combination with lung involvement, is considered to be the most important determinant of the prognosis of these patients. Cardiac involvement occurs in 15– 35% of cases but in autopsy surveys the incidence reaches 80%. The case we present concerns a woman of 65 years, admitted in December at the Department of Cardiology of our AOU for an episode of low–output heart failure with epigastralgia, sweating, and breathlessness. The cardiological history was almost silent; instead, it presented a history of chronic thyroiditis and a recent infection with SARS COV 2. The objective examination presented hypokinesis of basal pulmonary fields and pitting edema. The blood tests showed an increase in myocardial necrosis, BNP, creatininemia and inflammatory indices (VES, PCR, beta 2 microglobulin, ferritin, fibrinogen), normocytic normochromic anemia, hypogammaglobulinemia, hyperuricemia and proteinuria and hematuria. The echocardiogram showed severe biventricular dysfunction and moderate–grade mitral insufficiency. A coronary examination was performed that showed epicardial coronary vessels free from significant injury. The cardiac magnetic resonance showed a widespread late enhancement subendocardial of the ventricular and atrial chambers with non–vascular distribution suggestive of pathology on an autoimmune basis. Upon closer examination, multiple small telangiectasias were observed in the face, slight hyperpigmentation of the chest and back, and swelling of the hands. The antibody panel showed positivity of ANA and anti Scl70. The patient was thus addressed to a multi–specialist management in which therefore cardiologist and rheumatologist compare and support the treatment of this pathology with the goal of reducing the high mortality and morbidity that characterizes systemic scleroderma with cardiac involvement.

Multiple cutaneous telangiectasias after the use of trastuzumab: a case report

Multiple cutaneous telangiectasias after the use of trastuzumab: a case report

Dental manifestations of hereditary hemorrhagic telangiectasia: a clinical case

A rare disorder that changes the blood vessels of many organs and causes bleeding. The disease affects the lungs, liver, central nervous system, gastrointestinal tract, skin and mucous membranes. The degree of incidence of Randu-Weber-Osler disease is not high and ranges from 1 detected case per 50 thousand – per 100 thousand population. The scientific article presents a clinical case with dental manifestations of Randu-Weber-Osler disease and indicates the important role of the dentist in monitoring such patients. The red border of the lips is dry, pale, with pronounced multiple punctate telangiectasias of red-violet color. In the area of the lower lip, brightly colored round nodules with a diameter of about 5 mm are found, protruding above the surface, painless on palpation. On the dorsal and lateral surfaces, there are papular formations of a red-violet color, with a diameter of 5 to 7 mm, protruding above the surface. The gums are hyperemic, edematous, loose, bleeds on probing, periodontal pockets with exudative contents. On examination, attention was drawn to an insufficient amount of saliva, which was located mainly in the retromolar region; it was frothy. The salivation rate is reduced (0.1 ml per minute). It is assumed that before and during invasive dental procedures, antibiotic therapy is necessary, especially when working in infected areas (500 mg of amoxicillin) every 8 hours starting 12 hours before the procedure for 7 days. Given that hypoxia and cerebral embolism are frequent complications, the dental chair should be in an upright position throughout the patient's appointment. Treatment of this disorder is only supportive in nature, its goals are to relieve symptoms, treat complications and prevent progression of the disease. Early definition of the manifestations of this disease and careful observation of the patient gives a favorable prognosis of the course and prevents the development of severe complications. Keywords: hereditary hemorrhagic telangiectasia, periodontitis, xerostomia.

Hereditary Haemorrhagic Telangiectasia of Nasal Mucosa- A Case Report

Hereditary Haemorrhagic Telangiectasia (HHT) is also known as Osler-Weber-Rendu syndrome is a development disorder of blood vessels. It is characterised by multiple telangiectases and Arteriovenous Malformations (AVMS) that cause direct connections between arteries and veins bypassing intermediate capillaries. The most prominent sites for telangiectasia are the lips, tongue, face, mucosa of the nose, oral cavity, and gastrointestinal tract. Telangiectases can rupture and bleed easily due to their thin walls, narrow tortuous courses, and proximity to the skin's surface or a mucous membrane. Depending on the organ affected, these symptoms include repeated persistent epistaxis, skin lesions, haemoptysis, gastrointestinal haemorrhage, and stroke. Complications of HHT include bleeding, anaemia, followed by stroke, pulmonary AVM, Transient Ischaemic Attack (TIA), hypovolemic shock due to severe intractable bleed, eventually leading to high output failure. Present case is of a 45-year-old female who presented with complaints of recurrent epistaxis. Diagnostic nasal endoscopy showed multiple telengiectasic spots which were cauterised with trichloroacetic acid. However recurrent episodes of bleed were encountered.

A case of a long course of Osler–Weber–Rendu disease in a 65-year-old patient

The incidence of Osler–Weber–Rendu disease is low, ranging from 1 detected case per 50,000 to 1 per 100,000 population. The disease is hereditary, with autosomal dominant transmission, caused by pathogenic mutations in genes involved in angiogenesis. The disease has a pronounced clinical picture of multiple telangiectasias of the skin and mucous membranes and manifests as spontaneous bleeding. This scientific article presents a clinical analysis of a 65-year-old patient with a diagnosis of Osler–Weber–Rendu disease. Early identification of the manifestations of this disease and careful observation of the patient give a favorable prognosis of the course and prevent the development of severe complications.

Bleeding Risk Following Stereotactic Body Radiation Therapy for Localized Prostate Cancer in Men on Baseline Anticoagulant or Antiplatelet Therapy

PurposePatients on anticoagulant/antiplatelet medications are at a high risk of bleeding following external beam radiation therapy for localized prostate cancer. SBRT may reduce the bleeding risk by decreasing the volume of bladder/rectum receiving high doses. This retrospective study sought to evaluate the rates of hematuria and hematochezia following SBRT in these patients.MethodsLocalized prostate cancer patients treated with SBRT from 2007 to 2017 on at least one anticoagulant/antiplatelet at baseline were included. The minimum follow-up was 3 years with a median follow-up of 72 months. Patients who had a rectal spacer placed prior to SBRT were excluded. Radiotherapy was delivered in 5 fractions to a dose of 35 Gy or 36.25 Gy utilizing the CyberKnife system. Hematuria and hematochezia were prospectively assessed before and after treatment using the Expanded Prostate Cancer Index Composite (EPIC-26). Toxicities were scored using the CTCAE v4. Cystoscopy and colonoscopy findings were retrospectively reviewed.ResultsForty-four men with a median age of 72 years with a history of taking at least one anticoagulant and/or antiplatelet medication received SBRT. Warfarin (46%), clopidogrel (34%) and rivaroxaban (9%) were the most common medications. Overall, 18.2% experienced hematuria with a median time of 10.5 months post-SBRT. Altogether, 38.6% experienced hematochezia with a median time of 6 months post-SBRT. ≥ Grade 2 hematuria and hematochezia occurred in 4.6% and 2.5%, respectively. One patient required bladder neck fulguration and one patient underwent rectal cauterization for multiple non-confluent telangiectasia. There were no grade 4 or 5 toxicities. Cystoscopy revealed bladder cancer (40%) and benign prostatic bleeding (40%) as the most common hematuria etiology. Colonoscopy demonstrated hemorrhoids (54.5%) and radiation proctitis (9.1%) as the main causes of hematochezia. There was no significant change from the mean baseline EPIC-26 hematuria and hematochezia scores at any point during follow up.ConclusionIn patients with baseline anticoagulant usage, moderate dose prostate SBRT was well tolerated without rectal spacing. High grade bleeding toxicities were uncommon and resolved with time. Baseline anticoagulation usage should not be considered a contraindication to prostate SBRT.

Atypically located spider angioma

To the Editor,
 A spider angioma or spider naevus is a type of telangiectasis found slightly beneath the skin surface, often containing a central arteriole and many small vessels which radiate outwards like a spider's web. Applying pressure to the center of the lesion leads to fading of the lesion. Spider angioma is a common presentation of liver cirrhosis, especially alcoholic cirrhosis. Spider angiomas are found only in the distribution of the superior vena cava, and are thus commonly found on the face, neck, upper chest and upper arm. Spider angioma is rarely observed on hand (1, 2). Here, we report a a very rare case with spider angioma on back of hand.
 A 54-year-old male patient admitted to our clinic with icterus and ascites. The patient had a diagnosis of alcoholic liver cirrhosis one month before. On physical examination, he had icterus and ascites. He had spider angiomas on his back, neck and on the back of his right hand, just proximal to second metacarpophalangeal joint (Figure 1). The patient’s Child Turcotte Pugh score (CTP) and model for end stage liver disease (MELD) scores were 12 and 22, respectively. Abdomen ultrasound showed cirrhosis with massive ascites. Endoscopy revealed grade 1 esophageal varices and portal hypertensive gastropathy. The patient gave written consent regarding this article.
 Spider angioma appears frequently in alcoholic cirrhotic or noncirrhotic alcoholic liver diseases with deuteriation of liver functions. Although, the exact cause of SA is not known, there have been many theories of possible etiologic factors such as disturbance of sex hormones (increased level of luteinizing hormone, decreased level of testosterone and a higher estradiol/testosterone ratio), angiogenesis, vasodilation, alcohol abuse, hyperdynamic circulation state, and liver dysfunction[3, 4]. The number and size of SA are correlated with the degree of liver disease (1, 3, 4). 
 Our patient was a heavy drinker and had alcoholic liver cirrhosis with high CTP and MELD score. Although, our patient had multiple telangiectasias, it is a very rare finding of SA, observed on back of hand.

S3599 A Rare Case of Resistant H. pylori-Associated With GI Telengiectasia

INTRODUCTION: Helicobacter Pylori (H. Pylori) is a commonly identified pathogen in cases of dyspepsia. The treatment involves initial eradication, with salvage therapies reserved for refractory cases. Here we report a case of difficult to treat H. Pylori infection associated with gastrointestinal telangiectasias. CASE DESCRIPTION/METHODS: A 20-year-old male presented to our clinic with complaints of persistent dyspepsia, heartburn, and 4 kg weight loss for the last 3 months despite taking proton pump inhibitors (PPI). Initial blood tests were normal. An endoscopy was performed which showed hyperemic mucosa in the body and antrum of the stomach along with multiple telangiectasias in fundus and body of the stomach (Figure 1). Biopsies were performed which showed H. Pylori infection and chronic active gastritis. The patient denied any blood in stools and there was no similar problem in the family as well. The patient was given a two weeks course of PPI, amoxicillin, and clarithromycin. His symptoms improved a little bit during the course. Two weeks after the completion of therapy, his stool for H. Pylori antigen was checked for the confirmation of eradication, which came back positive. He was then prescribed salvage therapy comprising of PPI, amoxicillin, and levofloxacin for 2 weeks. After 1 month of completion of eradication therapy, his stool for H. Pylori antigen was re-checked, however, it again came back positive. He is on high dose PPI and his symptoms have improved, but not completely resolved. DISCUSSION: H. Pylori is a frequent infective cause of gastrointestinal pathologies. The initial eradication therapy involves a combination of PPI and antibiotics. For refractory cases, salvage therapy is recommended. The two proposed regimens include bismuth-containing quadruple or triple therapy including levofloxacin. This case signifies the growing resistance to available therapies and the need to look for new treatment options.Figure 1a.: A single telengiectasia seen in middle third of esophagus b. Telengiectasia seen in body of stomach c. Multiple small sized telengiectasias seen in the gastric fundus.

Epistaxis e Hemorragia Digestiva Recorrente: Manifestação da Síndrome Osler-Weber-Rendu

A telangiectasia hemorrágica hereditária ou síndrome Osler-Weber-Rendu é uma doença vascular hereditária autossómica dominante, que se apresenta, geralmente, com telangiectasias mucocutâneas, epistaxe, hemorragia gastrointestinal, anemia ferripriva e malformações arteriovenosas.Os autores apresentam o caso clínico de um doente, género masculino, 78 anos, com insuficiência cardíaca descompensada, anemia ferripriva com necessidade transfusional e múltiplas telangiectasias. Nos antecedentes, destacava-se uma história de epistaxes espontâneas e recorrentes desde a juventude e episódio de hemorragia digestiva recente. Durante o internamento, apresentou epistaxe e hematoquézias, complicados com choque hipovolémico. Foi admitido o diagnóstico de síndrome de Osler-Weber-Rendu, tendo iniciado hormonoterapia. O internamento prolongou-se por complicações hemorrágicas e infeciosas, tendo o doente falecido ao 30º dia em edema agudo do pulmão.O caso apresentado corresponde a síndrome de Osler-Weber-Rendu, com critérios clínicos, apresentando uma evolução desfavorável.

Sequence variations of ACVRL1 play a critical role in hepatic vascular malformations in hereditary hemorrhagic telangiectasia

BackgroundHereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder characterized by multiple telangiectases and caused by germline disease-causing variants in the ENG (HHT1), ACVRL1 (HHT2) and, to a lesser extent MADH4 and GDF2, which encode proteins involved in the TGF-β/BMP9 signaling pathway. Common visceral complications of HHT are caused by pulmonary, cerebral, or hepatic arteriovenous malformations (HAVMs). There is large intrafamilial variability in the severity of visceral involvement, suggesting a role for modifier genes. The objective of the present study was to investigate the potential role of ENG, ACVRL1, and of other candidate genes belonging to the same biological pathway in the development of HAVMs.MethodsWe selected 354 patients from the French HHT patient database who had one disease causing variant in either ENG or ACVRL1 and who underwent hepatic exploration. We first compared the distribution of the different types of variants with the occurrence of HAVMs. Then, we genotyped 51 Tag-SNPs from the Hap Map database located in 8 genes that encode proteins belonging to the TGF-β/BMP9 pathway (ACVRL1, ENG, GDF2, MADH4, SMAD1, SMAD5, TGFB1, TGFBR1), as well as in two additional candidate genes (PTPN14 and ADAM17). We addressed the question of a possible genetic association with the occurrence of HAVMs.ResultsThe proportion of patients with germline ACVRL1 variants and the proportion of women were significantly higher in HHT patients with HAVMs. In the HHT2 group, HAVMs were more frequent in patients with truncating variants. Six SNPs (3 in ACVRL1, 1 in ENG, 1 in SMAD5, and 1 in ADAM17) were significantly associated with HAVMs. After correction for multiple testing, only one remained significantly associated (rs2277383).ConclusionsIn this large association study, we confirmed the strong relationship between ACVRL1 and the development of HAVMs. Common polymorphisms of ACVRL1 may also play a role in the development of HAVMs, as a modifying factor, independently of the disease-causing variants.

A CASE OF OSLER WEBER RENDU SYNDROME AND ITS ANAESTHETIC IMPLICATIONS

Osler-Weber-Rendu disease or hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder. First recognised in the 19th century, this rare often undiagnosed familial disorder with abnormal vascular structures, causes bleeding from the nose and gastrointestinal tract. This condition is characterised by lack of communicating capillaries connecting arteries and veins resulting in multiple arteriovenous malformations (AVMs) and telangiectasia. We describe a case report of anaesthesia management of a patient with Osler-Weber-Rendu disease.

The applicability of Sonic hedgehog in mixed type basal cell carcinoma

Background : Basal cell carcinoma (BCC) is the most common skin malignancy found in nonmelanoma skin cancer. There are multiple factors contribute to risks for BCC but the underlying mechanism is genetic alteration. The Sonic hedgehog (SHH) pathway is implicated in the etiology of the most common BCC. We reported BCC nodular type in the tip of nasal region with increase SHH. Case Illustration: 50 years old man, presented with hyperpigmented tumor on the nasal tip which easily bleed since 6 months ago. Dermatological examination: the tip of nasal region: a solitaire irregular immobile hyperpigmented tumor, with the size of 2x1x0,3 cm, pearly border with rubbery consistency. Dermoscopy examination found multiple blue-gray globule and telangiectasia feature. Histopathology examination found nodule and micronodule with palisade feature in the periphery and “slit like retraction” in central. Immunohistochemistry examination found more than 50% cell show SHH expression. Wide surgical excision was done with tumor margin 3 mm. Discussion: The hedgehog (Hh) pathway known have important role in embryonic development, that responsible for patterning development organ and tisuue. SHH is one protein ligand that most study and well known in Hh pathways. Conclusion : Activating SHH pathway can resulting in unrestricted SMO activity that causes uncontrollable cell proliferation and eventually carcinogenesis. Increased expression of SHH can be used as a marker in aggresivity of BCC. Keywords: clinical manifestation, histopathology, immunohistochemistry, Sonic hedgehog pathway

Case of Osler-Weber-Rendu Syndrome Complicated with Nasal Septum Perforation

Objectives: Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is a rare autosomal dominant disorder characterized by multiple mucocutaneous telangiectasias and visceral arteriovenous malformations. We report the first case of Randu-Osler-Weber disease complicated with nasal septum perforation. Method: Diagnosis was based on physical examination and genetic testing. Results: A 52 years old female presented to the Mongol-Japan Teaching Hospital for evaluation of anemia and recurrent epistaxis. On physical examination, telangiectasias were found on eye mucous, tongue tip, palms, and fingers. An ACVRL 1 mutation was detected by genetic testing. Conclusion: On the basis of clinical features in this patient telangiectatic lesions in nasal cavity lead to epistaxis which is more common than mucocutaneous telangiectasia in HTT. Recurrent nose bleeding leads to iron deficiency anemia.

Case report on hereditary haemorrhagic telangiectasia

<p class="abstract">Osler Weber Rendu disease also known as hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries resulting in direct connections between arteries and veins. A 70-year-old male patient known case of hypertension; Indian origin was brought to the emergency department of our hospital with complaints of severe epistaxis. An attempt was made initially to control bleeding by ice compressions and finally anterior and posterior nasal packing was done. He gave history of similar episodes in the past often accompanied with hemetemesis and malena on and off since childhood gastroduodenoscopy was done to rule out cause for hemetemesis which showed multiple telangiectasia in fundus, body and antrum of stomach and first part of duodenum establishing the diagnosis of HHT. Patient with Osler-Weber–Rendu disease may present with uncontrolled bleeding. Resuscitation along with hemostasis is the main stay of treatment. As the bleeding occurs from malformed vessels coagulation tests are normal. Management include blood transfusion, antifibrolytics and surgical hemostasis.</p>

A CASE REPORT ON OSLER-WEBER-RENDU DISEASE

Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membrane and in organs such as lungs, liver, and brain. Here, we report the case of a 68-year-old postmenopausal woman who was admitted to our hospital following multiple episodes of hematemesis and melena during the past 5 days. Upper GI endoscopy revealed multiple telangiectasia with active spurting and gastric polyp. The patient was treated with 1 unit of packed red blood cells transfusion for anemia, Inj. Tranexamic acid 500 mg IV tid and Inj. Ondansetron 4 mg IV for hematemesis. Tab. Thalidomide 100 mg has been given for treating hereditary hemorrhagic telangiectasia. The treatment of HHT is only palliative, with no consensus on the best treatment option. It is essential to promote control of thedisease as long as possible.

Positive Antinucleolar Antibodies on Direct Immunofluorescence of Lesional Skin of a Patient With Limited Systemic Sclerosis

To the Editor: Limited systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs. Circulant anticentromere antibody is usually positive.1 Antinucleolar antibodies are positive less frequently (15%–40%),2 having prognostic significance on the overall course of the patients. We present a case of limited SSc with negative serologic test for extractable nuclear antigens in which direct immunofluorescence revealed positive antinuclear antibodies (ANA) with nucleolar pattern. A 54-year-old man was referred to the dermatology department because of a 5-year history of multiple asymptomatic telangiectasias on the face and neckline. Clinical examination revealed the presence of sclerodactyly and multiple scars of ulcers in the fingertips that appeared when the patient worked in a frigorific chamber. Capillaroscopy showed predominance of areas of angiogenesis and some small avascular areas. The patient complained about mild interphalangeal arthralgias without inflammatory changes. Unspecific gastrointestinal symptoms and dyspnea were also present. High-resolution chest computed tomography showed mild pulmonary fibrosis predominantly in peripheral areas and bases of the lungs. Echocardiography confirmed the clinical suspicion of pulmonary hypertension, and bosentan therapy was initiated. Circulant ANA resulted positive at 1:1280 dilution. The determinations of extractable nuclear antigens, antibodies against parietal cells, against smooth muscle, and against double-stranded DNA were all negative. A skin biopsy of the neckline area with telangiectasia revealed lymphocytic perifolliculitis, with no other alterations. Direct immunofluorescence study (DIF) performed on lesional skin showed the presence of IgG ANA with a nucleolar pattern (Fig. 1).FIGURE 1.: Direct immunofluorescence on a skin biopsy from an area with telangiectasia in the patient's neckline. IgG resulted positive with a nucleolar pattern (×100).Circulating autoantibodies are found in as many as 95% of patients with SSc and carry greater weight in the newly proposed ACR/EULAR classification system. Centromere (CEN) proteins (P), topoisomerase I, and RNA polymerase III autoantibodies closely reflect patterns of organ involvement and disease progression.3 Circulating anti-Th/To type antinucleolar antibodies are detectable mainly by immunoprecipitation, which is not performed routinely in most clinical laboratories. Its positivity is related to limited skin involvement and frequent fibrosis of internal organs (interstitial lung disease, pericarditis, and renal involvement). Frequency of negative circulating antibodies with positive DIF findings is largely unknown because the last is not routinely performed. In the study by Kulthanan et al,4 a subgroup of patients with SSc presented positive DIF findings in 77% of the cases with the dermoepidermal junction and epidermal nuclei as the most common site, and of these patients, 45% presented negative serum ANA. Shibeshi et al5 reported a DIF positivity rate of 25,7% in 63 patients with SSc, with only one case with no circulating autoantibodies at all, and another with positive serum ANA but no characteristic antibodies of SSc. Antinucleolar pattern has been rarely reported in DIF of patients with SSc. Only 6 cases were found in the literature, all of them with high ANA titles. As these cases date from the 1980s, only circulating anti-ribonucleoprotein was performed, resulting positive in one of the patients.6,7 Our patient had ANA in peripheral blood, but more specific antibodies were not detected (antinucleolar, Scl70, or anticentromere). However, a skin biopsy with DIF was allowed to demonstrate the fixation of antibodies with a nucleolar pattern. Nucleolar pattern ANA in DIF of a skin biopsy is an unusual finding. We believe this technique can help to confirm clinical diagnosis and assess prognosis on individual patients.

SPLENIC ABSCESS AND PERISPLENIC HEMATOMA COMPLICATING OSLER

A 52-year-old diabetic gentleman was referred to our center with a 3-week history of fever, left-sided abdominal pain, and progressive breathlessness. He also had history of recurrent epistaxis since childhood. Contrast-enhanced computerized tomography chest and abdomen revealed a splenic abscess, left pulmonary arteriovenous malformation, and left pleural effusion. He was managed conservatively with intravenous antibiotics and an antifungal. A repeat imaging was done after 3 weeks which showed resolution of abscess but an increase in the size of the perisplenic hematoma. An ultrasound guided pigtail catheter was inserted into the peri-splenic hematoma and it was drained. He had also developed an acute cerebellar infarct detected on magnetic resonance imaging of brain, which also showed other chronic infarcts of varying age. A diagnostic nasal endoscopy revealed multiple telangiectasias, and Osler