AN EDITION OF GREEK RHEUMATOLOGY SOCIETY AND PROFESSIONAL ASSOCIATION OF RHEUMATOLOGISTS
In this case, we present a patient with unilateral salivary gland enlargement and periorbital edema with erythematous rash. We discuss the differential diagnosis and the relevant therapy. Mediterr J Rheumatol 2017;28(1):57-8 https://doi.org/10.31138/mjr.28.1.57 Article Submitted 21/09/2016; Revised form 28/11/2016; Accepted 13/12/2016 Corresponding author: Alexandros A. Drosos, MD, FACR, PhD Professor of Medicine/Rheumatology Rheumatology Clinic, Department of Internal Medicine Medical School of the University of Ioannina Ioannina 45110, Greece Tel.: +302651007503 Fax: +302651007054 E-mail: adrosos@cc.uoi.gr Dermatomyositis sine myositis – Case presentation Evripidis Kaltsonoudis, Eleftherios Pelechas , Alexandros A. Drosos Rheumatology Clinic, Department of Internal Medicine, Medical School University of Ioannina, Ioannina, Greece MEDITERRANEAN JOURNAL OF RHEUMATOLOGY 28 1 2017 58 2. Although Sjögren’s syndrome could manifest with parotid gland enlargement (unilateral or bilateral) as the first manifestation,3 in this case, the patient did not have xerostomia, xerophthalmia and also had a negative Schirmer’s test and a negative salivary gland biopsy. Finally, the laboratory workup did not show any specific autoantibodies. 3. In order to diagnose SLE, 4 out of 11 criteria should be met.4 In our case, the patient had (subjective) photosensitivity, and a (weakly) positive ANA titer only two of those criteria. Patients with Dermatomyositis are at times difficult to distinguish from patients with subacute cutaneous lupus erythematosus.5 4. Dermatomyositis sine myositis or amyopathic Dermatomyositis is a rare but distinct subtype of Dermatomyositis.6 It is diagnosed in patients with typical cutaneous manifestations (consisting of heliotrope rash, facial erythema and edema, Gottron’s papules and periungual telangiectasia) in whom there is no evidence of muscle weakness and who repeatedly have normal serum muscle enzyme levels. There is a female to male preponderance (3:1) and the onset of the disease usually occurs in early adulthood. Dermatomyositis sine myositis should be aggressively treated even in the absence of muscle involvement since intense and prolonged skin inflammation can result in cutaneous ulceration and calcinosis. Treatment is based on systemic immunosuppressive therapy (high dose corticosteroids, methotrexate, azathioprine, mycophenolate) or immunomodulatory therapy (high dose intravenous immunoglobulins). In the presented case, the patient was treated with high dose of methylprednisolone (32mg) once a day along with calcium and vitamin D supplements. A month later, the patient showed significant improvement of the rash (Figure 1 right). In addition, she did not develop muscle weakness or muscle enzyme abnormalities. In conclusion, DsM, even a rare condition, should be a differential to be borne in mind for clinicians because it needs an aggressive treatment in order to prevent chronic skin changes and systemic complications such as pulmonary involvement. Finally, a close observation of the patient is mandatory, as DsM has been associated with different types of malignancies. CONFLICT OF INTEREST The authors declare no conflict of interest.