Rhupus syndrome: a case report Yesim Akpinar 1 ✉ , Ülker Karagece Yalcin 2 1 Department of Dermatology, Ufuk University, Ankara, Turkey. 2 Department of Pathology, Gören Private Clinic, Ankara, Turkey. ✉ Corresponding author: yesim_akpinar@yahoo.com 77 2017;26:77-79 doi: 10.15570/actaapa.2017.24 Introduction Autoimmune diseases are known as familial chronic and systemic inflammatory disorders that can be characterized by immune sys- tem dysregulation. Rhupus syndrome is a rare disease that is clin- ically and immunologically observed together with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). The prev- alence of the disease is 0.09% (5). This syndrome’s prevalence among systemic rheumatic diseases is estimated to be between 0.01 and 2% (12). The prognosis in rhupus syndrome depends on internal organ damage, but it is better than SLE and worse than RA. RA is responsible for Th1-mediated inflammation, whereas SLE is associated with Th2-mediated pathologies (1). In this case study, we report the case of a 22-year-old male pa- tient that was followed with a diagnosis of RA for the past 3 years. In our opinion, it is appropriate and beneficial to report this case study because the patient shows SLE symptoms together with RA. Case report A 22-year-old male patient was admitted to the hospital with com- plaints of redness on the face, forehead, and nose, hair loss, and joint pain. According to his medical history, pain and swelling in his knees, heel, and fingers, and especially morning stiffness in his knee, had started 3 years before he was admitted to the hospital. His previous laboratory tests showed an erythrocyte sedimen- tation rate of 37 (normal 0 to 20), and C-reactive protein 12 (normal 0 to 6). The rheumatoid factor was 4 (normal 0 to 8), and anti- cyclic citrullinated peptide antibody (anti-CCP Ab) 135.9 (normal 0 to 20). Based on the laboratory results and typical clinical pres- entation, the patient had been diagnosed with RA 3 years earlier. When the patient was admitted to our clinic, he was using ster- oids (prednisolone 5 mg/day). The examination of the patient was conducted in line with the Declaration of Helsinki principles. Physical examination findings The patient had butterfly-style erythema on the forehead, nose, and region that contains the malar projections (Figs. 1–2). He also had erythematous papules in some areas of the inner part of the leſt arm. There were alopecia plates of different diameters on various areas in the occipital scalp region (Fig. 3). There was tenderness in both knees and foot joints, and particularly in the heels, ac- cording to his joint examination. Cardiovascular, respiratory, and gastrointestinal examinations were normal. Laboratory findings Whole blood glucose, serum urea and creatinine levels, liver func- tion tests (ALT, AST), and rheumatoid factor were normal. There was no fecal occult blood. Sedimentation was 35 (normal 0 to 20), C-reactive protein was 15 (normal 0 to 6), antinuclear antibody (++fine granular ap- pearance), anti-dsDNA antibody (+++positive), antinucleosome antibody (+++positive), anti-histone antibody (++positive), anti- Smith antibody (++positive), proliferating cell nuclear antigen (PCNA) (+positive), anti SS-A/Ro (60kD) (++positive), anti-(U1)- ribonucleoprotein antibody (+positive), complement 3 (C3) 44.2 mg/dl (normal 20 to 88), and complement 4 (C4) 8.1mg/dl (normal 16 to 38) were normal, and HBsAg, anti-HBsAg, anti-HCV, and an- ti-HIV were negative. Serum iron (Fe) level was 18 µg/dl (normal 45 to 182 µg/dl). Electrocardiography and abdominal ultrasonog- raphy results were normal. There was no lytic or sclerotic lesion in other bone structures according to a bilateral two-way hand X-ray. Joint spaces and the surfaces of both knees and feet were normal according to a two- way X-ray. A skin biopsy was made from the erythematous plaque on the face. A skin punch biopsy showed mild acanthosis in the epi- dermis and in a few melanocytes in the dermis (HE ×200). There were polymorphous rich inflammatory cells in the environment of the dermal veins, and a few polymorphous and core parts on the small vein wall (Fig. 4). The patient was diagnosed with SLE and RA overlap disease according to the American College of Rheumatology (ACR) clas- sification criteria. The patient was given 200 mg hydroxychloro- quine per day. The butterfly-like rash improved aſter 18 days, but he still had pain. Therefore, 20 mg/day steroid treatment was ini- tiated. The dose of the steroid was gradually decreased to 5 mg/ day. The joint pain lessened on the 15th day of the steroid treat- ment. Remission was observed aſter 5 mg/day steroid and 200 mg hydroxychloroquine treatment. He was regularly followed up at 2-month intervals. Aſter 14 months of follow-up, he had no flare- up and he was in remission. Abstract Rhupus syndrome is a rare overlap syndrome that can be observed together with systemic lupus erythematosus (SLE) and rheu- matoid arthritis (RA). We report the case of a young male patient diagnosed with SLE due to new skin findings and also diagnosed with RA 3 years earlier. Keywords: rheumatoid arthritis, systemic lupus erythematosus, rhupus syndrome Acta Dermatovenerologica Alpina, Pannonica et Adriatica Acta Dermatovenerol APA Received: 21 April 2017 | Returned for modification: 31 May 2017 | Accepted: 21 June 2017