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Intraoral Telangiectasias Associated with Raynaud Disease: a Report of Two Cases Elizabeth Philipone, DMD1/Angela J

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Intraoral Telangiectasias Associated with Raynaud Disease: a Report of Two Cases Elizabeth Philipone, DMD1/Angela J QUINTESSENCE INTERNATIONAL Intraoral telangiectasias associated with Raynaud disease: A report of two cases Elizabeth Philipone, DMD1/Angela J. Yoon, DDS, MPH, MAMSc2/ David Zegarelli, DDS3 The finding of intraoral telangiectasias in two patients previously diagnosed with Raynaud disease is reported. Neither patient exhibited any other feature of CREST syndrome, name- ly, calcinosis cutis, esophageal dysfunction, or sclerodactyly. To the authors’ knowledge, this is the first time intraoral telangiectasias has been reported in conjunction with Raynaud disease in the absence of any other features of CREST syndrome. (Quintessence Int 2010;41:17–20) Key words: CREST syndrome, intraoral telangiectasias, Raynaud disease Telangiectasias are small, superficial, dilated CASE PRESENTATION blood vessels. Occurrence in the oral cavity is associated with two syndromes: CREST syn- Case 1 drome (calcinosis cutis, Raynaud disease, A 75-year-old woman was referred for evalua- esophageal dysfunction, sclerodactyly, and tion of a burning sensation on the anterior telangiectasia) and hereditary hemorrhagic palate and blue-red palatal spots. According to telangiectasia (HHT; Osler-Weber-Rendu syn- the patient, the burning sensation presented drome). An association with Raynaud disease 6 months prior, at which time she also in the absence of CREST has not been report- noticed the blue-red spots. The patient’s med- ed. Two patients with intraoral telangiectasias ical history was significant for high blood and Raynaud disease are presented. pressure, heart palpitations, and Raynaud disease. Her medications included diltiazem (240 mg/d) and nifedipine (60 mg/d). Intraoral examination revealed multiple small, red-blue, spiderlike lesions on the palate (Fig 1). A lesser number of similar papules were seen on the tongue and buccal 1Assistant Professor of Clinical Dentistry, Division of Oral and mucosa. The remainder of her intraoral exam- Maxillofacial Pathology, Columbia University College of Dental ination was normal. No clinical evidence of Medicine, New York, New York, USA. candida (to account for her complaint of 2Assistant Professor,Division of Oral and Maxillofacial Pathology, burning) was detected. An excisional biopsy Columbia University College of Dental Medicine, New York, New York, USA. of a palatal lesion was performed. Micro- 3Professor, Division of Oral and Maxillofacial Pathology, scopic evaluation revealed a series of dilated, Columbia University College of Dental Medicine, New York, thin-walled vascular channels lined by bland New York, USA. epithelium and containing a small number of Correspondence: Dr Elizabeth Philipone, Division of Oral and red blood cells. The vascular channels were Maxillofacial Pathology—PH 1562W, Columbia University College of Dental Medicine, 630 W. 168th St., New York, NY arranged in a linear pattern within and just 10032. Email: [email protected] below the lamina propria (Fig 2). VOLUME 41 • NUMBER 1 • JANUARY 2010 17 © 2009 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY. NO PART OF THIS ARTICLE MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER. QUINTESSENCE INTERNATIONAL Philipone et al Fig 1 Case 1. Clinical photograph Fig 2 Photomicrograph of a palatal lesion. demonstrating the presence of Multiple thin-walled blood vessels (arrows) close to small red macules of the palate. the overlying surface epithelium (hematoxylin- eosin; original magnification ϫ100). A diagnosis of ectatic vascular channels tongue. A sharp lingual cusp of the adjacent consistent with telangiectasia was rendered molar tooth was discovered; therefore, the for the palatal papule. It was noted in the tongue ulcer was thought to be traumatic in biopsy report that the telangiectasia might origin. The cusp was smoothed, and 0.05% be representative of the “T” in CREST. Other fluocinonide cream was prescribed. An exci- than Raynaud, the patient denied manifest- sional biopsy of a dorsal tongue lesion was ing any other classic symptom of CREST performed. Microscopic examination revealed syndrome. This was verified by her physi- multiple dilated, thin-walled vascular channels cian. Because no physical evidence of cause lined by bland epithelium and containing a for the burning sensation could be detected, small number of red blood cells (Fig 4). a concurrent diagnosis of burning-mouth A diagnosis of ectatic vascular channels syndrome was suggested. consistent with telangiectasia was rendered for the dorsal tongue. It was noted that the Case 2 vascular lesions might represent the “T” in A 53-year-old woman presented for evalua- CREST. Other than Raynaud, which had been tion of a painful sore on the left lateral side of diagnosed 18 years prior, the patient denied the tongue. The patient’s medical history was manifesting any other classic symptoms of significant for acid reflux, hypertension, CREST syndrome. When the patient present- Raynaud disease, and seasonal allergies. ed for follow-up, the tongue ulcer was signifi- Her medications included ibandronate sodi- cantly improved, supporting a traumatic origin um (150 mg per month), venlafaxine for the ulcer. hydrochloride (75 mg per day), omeprazole Because both patients were taking multi- (20 mg per day), nifedipine (60 mg per day), ple prescription medications, we briefly con- metoclopramide (40 mg per day), and mon- sidered the possibility that the lesions were telukast sodium (10 mg per day). medication-related. However, telangiectasia Intraoral examination revealed multiple development is not a reported adverse effect small, flat, blue lesions of the dorsal tongue of any of the medications either patient was and palate (Fig 3). A 5 ϫ 2-mm ulcer, lacking prescribed. This possibility was therefore induration, was present on the left lateral excluded. 18 VOLUME 41 • NUMBER 1 • JANUARY 2010 © 2009 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY. NO PART OF THIS ARTICLE MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER. QUINTESSENCE INTERNATIONAL Philipone et al Fig 3 Case 2. Clinical photo- Fig 4 Photomicrograph of a tongue lesion. graph demonstrating the pres- Multiple dilated, thin-walled blood vessels (arrows) ence of red-purple macules of the (hematoxylin-eosin; original magnification ϫ100). dorsal tongue (arrow). DISCUSSION Compounding the difficulty is that not all symptoms present simultaneously and they Telangiectasias are capillaries close to the can be separated by many years.1,2 Moreover, surface of a tissue or organ. Clinically, they patients with CREST may not manifest all five appear as multiple flat, small, red, nonpul- features.3 satile papules. Telangiectasias have been With the exception of Raynaud, both reported to occur in the oral cavity in associ- patients lacked any other feature of CREST. In ation with CREST syndrome and HHT.1 addition, neither patient demonstrated bleed- Histologic examination of telangiectasias ing, visceral involvement, or family history of reveals superficial clusters of dilated capillar- telangiectasias. Therefore, an association with ies.1 Biopsies taken from both patients HHT was excluded. In HHT, telangiectasias demonstrated multiple dilated, thin-walled present at an early age and involve the internal superficial blood vessels. In each case, the organs, as well as the oral and nasal mucosa, blood vessels were close to the mucosal sur- tongue, and lips. Epistaxis is often the initial face. Both cases were diagnosed as oral manifestation.2 telangiectasias. The clinical presentation, as Raynaud phenomenon is a condition in well as the microscopic appearance of both which exposure to cold causes vasospasm cases, was similar to the telangiectasias seen of the digital arteries, leading to an extreme in patients with CREST syndrome. blanching of the digits. When the hands are CREST syndrome is a form of limited scle- warmed, blood flow increases, thereby pro- roderma. The name is an acronym derived ducing a dusky-red appearance.1 The condi- from the clinical features of the syndrome: tion can be primary or related to systemic calcinosis cutis, Raynaud phenomenon, disease such as scleroderma or systemic esophageal dysfunction, sclerodactyly, and lupus. Primary occurrence is referred to as telangiectasia. The syndrome is most often Raynaud disease or primary Raynaud phe- diagnosed in women in the sixth or seventh nomenon, whereas secondary occurrence is decade of life.1 Diagnosis can be difficult referred to as Raynaud syndrome or second- because it is based on a compilation of clini- ary Raynaud phenomenon.4 Studies have cal, histologic, and immunologic findings. shown that Raynaud disease (primary VOLUME 41 • NUMBER 1 • JANUARY 2010 19 © 2009 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY. NO PART OF THIS ARTICLE MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER. QUINTESSENCE INTERNATIONAL Philipone et al Raynaud phenomenon) has an earlier age of REFERENCES onset than Raynaud syndrome (secondary Raynaud phenomenon).5 However, there 1. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral have been cases of Raynaud disease pre- and Maxillofacial Pathology, ed 3. St Louis: senting in patients over 40 years of age.5 Saunders, 2009. Those cases were categorized as late-onset 2. Ueda M, Abe Y, Fujiwara H, et al. Prominent telang- iectasia associated with marked bleeding in CREST Raynaud
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