Diagnosis and Treatment of Dermatomyositis-Systemic Lupus Erythematosus Overlap Syndrome Preston Williams1; Benjamin McKinney, MD2 1Texas A&M College of Medicine; 2Baylor University Medical Center Family Medicine Residency Introduction Case Description Discussion Dermatomyositis is an autoimmune condition classically A punch biopsy of her rash showed atrophic epithelium with This case of overlap syndrome between dermatomyositis and characterized by symmetric proximal muscle weakness, dyskeratotic keratinocytes, vacuolar interface changes, superficial systemic lupus erythematosus presents a rare but important inflammatory muscle changes, and dermatologic abnormalities.1 perivascular and lichenoid inflammation, and pigment challenge to the primary care physician. Our patient presented Several studies have shown that the inflammatory myopathies, incontinence consistent with systemic lupus erythematosus initially with arthralgias and fatigue, symptoms more such as dermatomyositis, commonly overlap with other (SLE). The patient was started on prednisone 40 mg daily for a 2- characteristic of systemic lupus erythematosus. However, these connective tissue disorders, significantly complicating the week taper to 10 mg and hydroxychloroquine 200 mg daily with symptoms were followed by a facial rash that involved the diagnosis.2 The reported incidence of overlap syndrome ranges marked improvement in symptoms. Further lab work-up was nasolabial folds and periorbital regions more in line with from 11% to 40% in patients diagnosed with significant for a positive SSA/RO but an otherwise negative dermatomyositis. Interestingly, despite the prominence of the dermatomyositis. Overlap syndromes in dermatomyositis are antibody panel. Upon tapering of her prednisone, the patient was characteristic proximal muscle weakness, a rash is the most most common with connective tissue diseases such as Sjogren subsequently hospitalized for acute worsening of her initial common presenting feature of dermatomyositis.4 This patient’s syndrome, scleroderma, rheumatoid arthritis, systemic lupus symptoms with profound muscle weakness and peripheral edema. initial labs were also not significantly concerning on presentation erythematosus, and mixed connective tissue disease. A disorder She also developed progressive dysphagia, left sided ptosis, and as her muscle enzymes didn’t show marked elevation until the can only be considered an overlap syndrome if the patient meets worsening of her rash with normal acute brain imaging. The patient had already developed significant disease. The atypical the separate diagnostic criteria for both conditions, however, creatinine kinase was 2,775 U/L, aldolase 14.8 U/L, aspartate mix of symptoms with relatively benign laboratory findings overlap syndromes create diagnostic difficulty because the patient aminotransferase of 452 U/L, and alanine aminotransferase of exemplifies the difficulty in identifying overlap syndromes of often presents progressively with variable symptoms from each 120 U/L. Electromyography revealed a diffuse irritative classic rheumatologic conditions. Systemic glucocorticoids are disorder making it hard to establish a definitive disease process.3 myopathic process with significant decrement on repetitive the mainstay of initial treatment for dermatomyositis and are stimulation, consistent with dermatomyositis. Her magnetic often used in the treatment of SLE as well, suggesting the Case Description resonance imaging of the cervical spine showed diffuse, combination may be effective in dermatomyositis- SLE overlap.5 symmetric hyperintensity and edema in the muscles throughout However, prolonged use of glucocorticoids can result in steroid- A 39-year-old woman with known non-alcoholic fatty liver the cervical region, suggestive of inflammatory polymyositis. The induced myopathy, which can mimic the weakness of disease, iron deficiency anemia, and peripheral venous patient’s dose of prednisone was raised to 60 mg daily. She dermatomyositis as in the case of our patient. Ultimately, the insufficiency presented for the evaluation of a facial rash, improved and regained muscle strength but regressed after a 50- switch to tofacitinib improved the patient’s weakness and arthralgias, and worsening lower extremity edema. The day course of prednisone due to steroid-induced myopathy. She decreased the glucocorticoid dose needed.6 In our patient the arthralgias and edema had been present intermittently for one was tapered down on prednisone, initiated on mycophenolate and combination of tofacitinib, hydroxychloroquine, and low dose year but worsened over the past month. She reported no history of discharged with persistent weakness after a 2.5-month prednisone has been the most effective in inducing symptom a rash and had already tried some over the counter moisturizers hospitalization. She regained the ability to walk unassisted in remission in dermatomyositis and lupus overlap. without relief. Physical exam revealed 1+ bilateral edema to the rehab but continued to have hair loss, arthralgias, myalgias and a mid-calves and an erythematous, maculopapular rash across the diffuse rash of her upper extremities, chest and face. References nose, forehead, and behind the ears. The rash was initially treated Mycophenolate was stopped and switched to tofacitinib 11mg with hydrocortisone 2.5% and then ketoconazole 2% with no daily while she was continued on hydroxychloroquine 200 mg 1. Jablonska S, Blaszyk M. Scleromyositis (scleroderma/polimyositis overlap) is an entity. daily. Since the addition of tofacitinib the patient has experienced Journal of the European Academy of Dermatology and Venereology. 2004;18(3):265-266. improvement and her peripheral edema persisted despite the use 2. Tymms KE, Webb J. Dermatopolymyositis and other connective tissue diseases: a review of compression stockings and 20 mg of daily furosemide. The substantial improvement with regained muscle strength, hair of 105 cases. J Rheumatol. 1985; 12:1140. regrowth, resolution of her rash, and minimal arthralgias. Over 3. Koler RA, Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov patient followed up in the clinic 3 months later with worsening 1;64(9):1565-1573. lower extremity edema and facial rash, along with new, tender, six months of follow up, her symptoms remained in remission 4. Kovacs SO, Kovacs SC. Dermatomyositis. J Am Acad Dermatol. 1998;39:899–920. subcutaneous nodules in the abdomen, axillae, and groin. Initial and her dose of prednisone was able to be tapered down from 40 5. Guidelines/Outcomes Committee, Task Force. Guidelines of care for dermatomyositis. J Am Acad Dermatol. 1996;34(5):824-829. work-up showed a c-reactive protein of 2.2, erythrocyte mg to 2.5 mg daily. 6. Moghadam-Kia S, Charlton D, Aggarwal R, Oddis C. Management of refractory sedimentation rate of 87, a positive anti-nuclear antibody 1:1,280 cutaneous dermatomyositis: potential role of Janus kinase inhibition with tofacitinib. Rheumatology. 2019;58(6):1011-1015. in a speckled pattern, and microcytic anemia. Acknowledgement: This is derived in part from an article published in Baylor University Medical Center Proceedings, available online: http://www.tandfonline.com/ DOI:10.1080/08998280.2020.1821589.