‘Tho Korean Journal of Internal Medicine: 18:196-198, 2008 Dermatomyositis Developing in the first Trimester of Pregnancy In Whee Park, M.D., Yu Jin Suh, M.D., Jae Ho Han, M.D.*, Yoo Seob Shin, M., Jeong Hee Choi, M.D., Hae Sim Park, MD. and Chang Hee Sun, M.D. Departments of Allergy and Aheumatology, Anatomic pathology ‘Ajou University School of Meaicine, Suwon, Korea Dermatomyositis in pregnancy is rare. Pregnancy may be a precipitating factor at the onset or may develop during the course of dermatomyositis, which would exacerbate disease actviy. In this study, we report a 22-year-old female patient who developed generalized skin rash and progressive muscle weakness in the twelfth week of pregnancy. She was diagnosed with dermatomyositis and underwent therapeutic abortion, due to the high felal mortalty rate of the disease when developed in the first timester. Her symptoms improved with treatment of intravenous immunoglobulin and a high dose of corticosteroids. Key Words : Dermatomyositis, Pregnancy INTRODUCTION Dermatomyositis is a rare autoimmune disease with an incidence rate of one per a population of one milion”. Only 14% of cases ooour during chid-bearing years, and only a few cases of dermatomyosits associated to pregnancy ‘complications have been reported®. Therefore there is Felatvely ite information conceming the matemal and feta ‘outcome. We report a patient diagnosed with dermatomyositis in the first trimester of pregnancy. This is, to our knowedge, the fist report of a patient with dermatomyosiis during pregnancy in Korea, who was successfully treated with intravenous immunoglobuin (VIG) and a high dose of corticosteroids after therapeutic abortion, CASE REPORT ‘A 22-year-old Korean woman in the tweith week of her ‘second pregnancy was referred ffom the dermatology department as she developed symptoms of generalized weakness and skin rash, She had a past history of spontaneous aborion, Fourteen weeks ago, she had noticed faclal sweling and edema in both lags. She also had a ‘2-week history of generalized myalgia and a 10-week history of generalized weakness. She had been bed-ridden for eight Weeks. On admission, she complained of anorexia, nausea, and generalzed itching. Physical examination of the patent revealed moderate facial and peribheral edema, a generalized erythematous rash with discoloration on the face, neck, trunk, buttock, both arms and knees, The typical Gottron's papules (Figure 1A), perunqual telangiectasia, and periorbital hetotope rash were also observed (Figure 18), Blood pressure was: 120/70 mmkig and puse rate was 128 beatsimin, Neurologic examination showed grade 2 proximal muscle weakness in bot arms and legs and remarkable muscle tendemess, Complete blood counts, electroyte, urinalysis and thyroid function test were normal. Erythrocyte sedimentation rate (ESR) was elevated to 65 mmitr, Muscle enzymes were elevated with creatine kinase at 245 IUL (normal, 30~180 IU, aspartate aminotransferase at 67 IUIL (normal, 5~40 IU, lactic detycrogenase at 422 IU/L (normal, 100~220 |UD. Antinuclear antibody ter was 1:80 (homogenous type, but anil-RNP, ant-dsDNA, and theumatoid factor were + Rocoved + February 3, 2003 + Recepte March 20,2008 + Corespendores to ? Charg-Hoe Suh, MD, PRD. Asttant Professor, Department of Alergy and Ahoumology, Acu Universty Schoo of Mein, ‘San, Wenchecr-dong, Pau, Suwon, 42-721, Korea Tal CGI-210-518, Fax: CSI-210-5154, E-mal : chaun@ouac krIn Whee Park, et ak Dermatomyestis Developing in the Fist Timester of Pregnancy 197 negative. Elecvomyosraphic examination showed bizarre high frequency discharges, repettive fbilations and a decrease in the amplitude and duration of the motor action potentials, Nerve conduction studies were normal. Skin biopsy revealed {an atrophic epidermis and tyrone degeneration of the basal keratinocyte associated with a few perivascular ymphooytc infitrations (Figure 2). There were areas of subepidermal fibrin depostion, Direct immunofuorescence was negalve. We could not cblain muscle biopsy due to the patient's refusal, We decided to have an abortion due to profound muscle Weakness and high fetal motally in patients that develop dermatomyositis in the first timester. She had mutple skin abrasions on the neck, biateral arms, knees, and buttocks, There was also a wound infection on the chest wall. We began therapy with intravenous and topical antibiotics, In addition, we began medication of IVIG (2 alka divided into 5 days) instead of steroid due to skin infection. She improved slowly and was discharged 10 days after IVIG therapy. On clischarge the patient was wel, wih improved lmb weakness, Her proximal muscle weakness was graded as 55, which was an improvement from 215 at admission. The skin wound and rash took longer to clear, and she no longer required topical treatment 30 days later. Therefore, we decided to start prednisolone therapy at 60 mg dally. She was treated with IMG (1 akg for 1 day) every 4 weeks repeatedly. After 8 weeks, muscle enzymes retumed to normal, and we reduced predrisolone to 30 mg dally. DISCUSSION Dermatomyositis is classified as idopatic inflammatory imyopatiy®, Women are affected twice as often as men and, in aduls, the peak incidence occurs in the sith decade, although all age groups may be afected”. Only a few cases of dermatomyositis have involved complications in pregnancy’ Consequently, there Is relatively litle information concerning the outcome of pregnancy. Fetal morbidly and morality are substanial and. seem to pal matemal disease act. Stith or necnatal death complicated pregnancies in 7 of 15 patients ith acive dsease®, Even in patients that have the disease under contol with treatment, pregnancy may resut in fetal death @ of 14) or prematury (3 of 14, There are no consistent fetal or placental abnormalities to explain the degree of morality and prematuriy. When disease onse sin the frst timester, fetal mortality is very high (83%), and even vith onset in the second or thd timester, the sk for premature deivery remains high athough no infant has died in such a orcumstance"®.Fity percent of previous reported cases of dermatomyostis began dung the fist timester of pregnancy", as in the present case. These data allow us to consider pregnancy as a prechtang factor atthe onset and4198 Tho Korean Joumal of Ital Medina: VoL. 18, No, 9, Soptomber, 2008, inthe exacerbation of dermatomyositis, and stronaly advises close follow-up of the mother for early detection of disease flare-ups and timely treatment®, The rapid response after ‘abortion in this patient suggests that pregnancy might have ‘triggered the disease. A hypothesis suggesting a role for viral infection in the development of dermatomyositis, and a decrease in the humoral response 10 certain viral antigens ‘during pregnancy has been suggested to provide the link between dermatomyositis and pregnancy”. Dermatomyositis responds to steroids but often becomes ‘leroid-resistant, and some patients find the steroid side ‘effects unbearable, In a double-blind study of patients with refractory dermatomyositis, IVIG_ improved not only muscle strength and_skin rash, but also the underlying. immuno- pathology". Repeated open muscle biopsies of patients ‘ho improved cinicaly have shown marked improvement in the muscle cytoarchitecture, including muscle fber dlameter, revascularizaton with increased number of capillaries per fiber, and reduction of inflammation and connective tissue", ‘The Improvement in strength does not last more than 4-8 ‘weeks, and repeated infusions are required periodcally to maintain ®, Some patients with dermatomyositis who had become unresponsive to steroids may respond again to prednisolone after IVIG infusion", Since dermatomyositis responds to steroids, IVIG therapy is best reserved for sleroid-resistant patients, for patients not adequately controlled with combinations of steroids and methotrexate or ‘azathioprine (as a thrd-iine add-on), and for patients who are immunodeficient or in whom steroids and immunosuppressants ‘are contraindicated. We repeated IMIG infusions every 4 to 6 ‘weeks for maintenance therapy. ‘Our case and previous data allow us to consider pregnancy {88 a precitating factor at the onset and in the exacerbation of dermatomyosiis* ®", Therefore complaints of muscle ‘weakness and fatigue should not be dismissed as symptoms pregnancy alone, Complaints of muscle weakness, fatigue, ‘skin rash and edema should be investigated by evaluation of muscle strength and determination of creatine kinase. Pregnancy associated with dermatomyositis should be ‘considered as a high-risk We believe that beter understanding of this assoclation, together with improvements in matemal {and fetal monitoring and care wil result in better prognosis for fetal outcome, REFERENCES 1) Banker BQ, Engel AG. The pojmysits and denmatonyostis syraromes: In Engel AG. Barker BO. ed. Mjaboy. 1st es. New York MeGran-H, 1385-1422, 1986 2) Phoro Goa A, Goldenberg J, Ara E, Perea AB, Camano L, Schmidt B.Jivenie dermatomycsts and pregnancy. 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