Dermatomyositis an association ofgingival telangiectases and anti Jo1L A Dourmishev A L Dourmishev and R A Schwartz The presence of gingival telangiectases is an unusual clinical finding in ID: 959047
Download Pdf The PPT/PDF document "Acta Dermatoven APA Vol 16 2007 No 2" is the property of its rightful owner. Permission is granted to download and print the materials on this web site for personal, non-commercial use only, and to display it on your personal computer provided you do not modify the materials and that you retain all copyright notices contained in the materials. By downloading content from our website, you accept the terms of this agreement.
Acta Dermatoven APA Vol 16, 2007, No 2 Dermatomyositis: an association ofgingival telangiectases and anti Jo-1L. A. Dourmishev, A. L. Dourmishev, and R. A. Schwartz The presence of gingival telangiectases is an unusual clinical finding in adults with der-matomyositis (DM). Patients with aminoacyl-tRNA synthetase autoantibodies express one or more ofthe following features: myositis, interstitial lung disease, mechanics hands, and capillary abnormali-ties (facial telangiectases and Raynauds phenomenon).Case report: A 45-year-old woman with a classic form of DM of ten years duration was evaluated.Clinical investigation revealed periorbital edema and violaceous erythema of the eyelids, Gottrons pap-ules of the fingers, Gottrons sign on the elbows and malleoli, a plantar fissured, hyperkeratotic, andtelangiectases. The patient fulfilled Bohan and Peters criteria for the clinical, histological, EMG, andbiochemical diagnosis of DM. Elevated titers of ANA (1:320) with a speckled pattern and anti Jo-1 anti-bodies were found in her sera by ELISA and Western blot. The recognition of subsets within the spectrum of DM characterized by certain clinical andserological features may be important. Because facial telangiectases are a recognized finding in thissyndrome.C a s e r e p o r t dermatomyositis,gingivalsyndrome B S T R A C TDermatomyositis (DM) is a heterogeneous group ofacquired,
multisystem, inflammatory diseases that af-clinical features and laboratory characteristics (1, 2). Thetypical features of oral mucosa in DM are erythema andedema, hemorrhage, vesicles, erosions or ulcers,superficial vessels (3, 4). Capillary abnormalities in theantigen (cytoplasmic histidyl-tRNA synthetase) werecal investigation in Japan (7). In this case report, we Acta Dermatoven APA Vol 16, 2007, No 2 val telangiectases and the presence of circulating anti-Jo-1 antibodies. We suggest that the two may be associ-for Jo-1 syndrome or antisynthetase syndrome. Webelieve that capillary abnormalities in the gingiva mayA 45-year-old Caucasian woman was admitted inDecember 1997 with a heliotrope rash of the face, andpain, stiffness, and weakness of muscles in the upperand lower extremities. The proximal muscle weaknessof her arms and legs progressed rapidly. She had diffi-culty elevating her arms above her head, combing herhair, raising her body from a sitting position, climbingstairs, or exiting a car. She had no lung or joint com-plains, fever, or signs or symptoms suggestive ofRaynauds phenomenon. There was no family historytreated for a long period for tinea pedis without effect.violaceous erythema of the eyelids, saturated wine-redcolored flat-topped papules overlying the skin cover-and scaling. Physical examination of the oral cavity re-vealed edema of the gingiva and
single dilated telang-iectases along the lower gingival margin abutting boththe dentition and interdental region and on the neigh-A complete blood count and routine chemistrieswere within normal ranges. The erythrocyte sedimen-tation rate was elevated (50 mm/h Westergreen), se-rum creatine kinase (CK) exceeded the normal ranges15-fold (1803 U/l), and ASAT and ALAT were high (104U/l and 112 U/l, respectively). Indirect immunofluores-�cence (IIF) for ANA on HEp-2 cells was positive ( 1:320)and demonstrated a speckled pattern. Antibodies to Jo-1 and SS-A52 kD antigens were detected by ELISA andWestern blot. The muscle biopsy specimen showedtrate with perifascicular and perivascular localization.pathology of skin biopsy specimens revealed anorthokeratotic, partly atrophic epidermis with focal basaldegeneration and pigment incontinence. Obvious thick-papillary edema and discrete lymphocytic infiltrationin the dermis.Initially the patient was treated with oral methyl-prednisolone, 80 mg daily in stepwise decreasing dosesmonth because of a relapse. From February 2002 thedisease was controlled with low-dose methotrexate (7.5mg per week) and 8 mg methylprednisolone, with im-proved muscle strength. However, the mucosal gingi-well-controlled muscle symptoms, but mucosal telang-talized again because of a relapse with muscle involve-ment and a periorbital rash, prompting treatment wit
hazathioprine 100 daily and intravenous methylpredniso-lone. She responded well, prompting reduction in15 mg prednisolone daily.muscles and cutaneous rash consisting of heliotropefirming the clinical, histological, EMG, and biochemi-were prominent in our patient. In 1939, Keining (9) firsttorted cuticles in a DM patient. In 1958, Gottron (10)appearance characterized by irregular overgrowth andtelangiectases, and hemorrhages.appearance of calloused feet. Usually they are diag-nosed incorrectly as having pityriasis rubra pillaris (PRP).soles, in whom a biopsy confirmed the clinical impres-sion of pityriasis rubra pilaris. Later, Christianson et al.(13) reported 270 patients with DM, including OLearysresembling PRP but without histological confirmation.In 1969, Wong (14) described 11 patients with general-C a s e r e p o r t Acta Dermatoven APA Vol 16, 2007, No 2 3. Ghali FE, Stein LD, Fine JD, Burkes EJ, McCauliffe DP. Gingival telangiectases. An underappreciatedtoimmunity. Nature. 1983;304:177 9.network of dilated superficial vessels (4). He also fo-cused on the prominent dusky red or bluish erythemaincluding gingiva, where there were closely set telang-margins, is frequently observed; however, edema with-out erythema was also noted. Vesicles, erosions, andoral cavity DM (3, 4, 20, 21). Leukokeratosis can reflectCapillary abnormalities in the gingiva have beendevelopme
nt of telangiectatic blood vessels scatteredthat these findings represented a systemic angiopathy(25 27), and that microvascular injury plays an impor-sions showed a significant degree of endothelial injury,its were found on the vessel walls of the dermis in ap-(28). Myositis-specific autoantibodies (MSA) are com-came apparent that MSA defined a group of myositispatients with distinctive clinical features (30). The pa-laries along the marginal gingiva, were associated withare associated with the presence of antibodies to histidylto HisRS and clinical manifestations of myositis, inter-Raynauds phenomenon were described as Jo-1 syn-drome or antisynthetase syndrome (30, 31). The termsdrome were introduced by Marguerie and Ray (32, 33).During the course of the syndrome, the patients withanti-Jo-1 autoantibodies expressed one or usually moresigns of a characteristic spectrum of various organ mani-arthritis, carpal tunnel syndrome, sclerodactyly, Sjögrenlary abnormalities (facial telangiectases and Raynaudsand mechanics hands in patients with DM do not re-(37). Our case could represent a coincidental associa-tive dermatomyositis. However, it may be presumed thattelangiectases on the gingiva, face, and trunk are dueto systemic microangiopathy, as far as they are evidentabnormalities in the disease.Recognition of subsets within the spectrum of DMcharacterized by the aforementio
ned clinical and sero-logical features is important in establishing a diagnosisand planning treatment, and is of prognostic signifi-festation of dermatomyositis with occult malignancyiectases in DM patients have recently been associatedC a s e r e p o r t E F E R E N C E S Acta Dermatoven APA Vol 16, 2007, No 2 C a s e r e p o r t34. Mitra D, Lovell CL, Macleod TI, Tan RS, Maddison PJ. Clinical and histological features of mechanics35. Dourmishev L, Wollina U, Hipler UC, Peytcheva V. Myositis-specific and myositis-associated autoan-tibodies in dermatomyositis and polymyositis. Clin Appl Immunol.36. Schmidt WA, Wetzel W, Friedlander R, Lange R, Sorensen HF, Lichey HJ et al. Clinical and serological37. Callen JP. Dermatomyositis. Lancet. 2000;355:53 7.38. Marton K, Hermann P, Danko K, Fejerdy P, Madlena M, Nagy G. Evaluation of oral manifestations and39. Healy CM, Tobin AM, Kirby B, Flint SR. Oral lesions as an initial manifestation of dermatomyositiswith occult malignancy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;101:184 7.Lyubomir A. Dourmishev MD, PhD, Department of Dermatology andVenereology, Medical University of Sofia, 1 St. Georgi Sofiiski Str., 1431 Sofia, Bulgaria, corresponding author, E-mail: l_dourmishevRobert A. Schwartz MD, MPH, Dermatology, New Jersey Medical School,185 South Orange Avenue, Newark, NJ 07103, USA,E-mail: roschwarcal.berkeley.edu