Rheumatology
Fazal-I-Akbar Danish in Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
Clinical:1 Joints: arthritis – non-erosive; involving >2 joints; deformities uncommon.2 Malar rash (butterfly rash) – fixed erythema across the nasal bridge and malar eminences.3 Discoid rash – circular lesions with red, raised margins and scarred, scaly centre; seen over face, neck and other sun-exposed areas.4 Photosensitivity.5 Mucous membranes: oral ulcers.6 CNS: fits; psychosis (without other cause).7 Cardiopulmonary: pleurisy; pericarditis.8 Haematological: haemolytic anaemia; leucopenia (<4 × 109/L); lymphopenia (<1.5 × 109/L); thrombocytopenia (<100 × 109/L).9 Renal: >0.5 g/day proteinuria; 3+ dipstick proteinuria; cellular casts.
Systemic Lupus Erythematosus 1
Len Sperry in Behavioral Health, 2013
There are, however, some common symptoms which characterize this condition: painful or swollen joints (arthritis), unexplained fever, and extreme fatigue, a characteristic red skin rash across the nose and cheeks, the so-called butterfly (malar) rash; chest pain upon deep breathing; unusual loss of hair; pale or purple fingers or toes from cold or stress (i.e., Raynaud’s phenomenon); sensitivity to the sun; swelling in legs or around eyes; mouth ulcers; swollen glands; and extreme fatigue. Other symptoms include chest pain, hair loss, anemia, and mouth ulcers. Some patients experience headaches, dizziness, depression, confusion, or seizures. New symptoms may continue to appear years after the initial diagnosis, and different symptoms can occur at different times (Sperry, 2009).
Sjögren syndrome and mixed connective tissue disease
Biju Vasudevan, Rajesh Verma in Dermatological Emergencies, 2019
Common cutaneous features include Raynaud phenomenon, periungual telangiectasias, acrosclerosis or sclerodactyly, swollen inflamed digits (“sausage fingers”), and symmetrically swollen hands (“puffy hands”). Raynaud phenomenon is the earliest skin manifestation and is present in almost all patients. Patients can develop painful digital ulcers, and even digital infarcts/gangrene in severe cases. Acute lupus-like features such as photosensitivity and malar rash can develop during disease flares [33–38]. Patients with MCTD often develop glandular features of Sjögren syndrome such as dry eyes and dry mouth (sicca complex) [39]. There may be well-defined sclerodermoid (unlike the diffuse binding down of skin in systemic sclerosis) or poikilodermatous (areas of mottled or reticulate dyspigmentation, telangiectasias, atrophy) lesions on the upper trunk and proximal limbs; however, other dermatomyositis-specific changes are usually absent. Oral ulcers and nasal septal perforation have also been described.
Initial hydroxychloroquine monotherapy in systemic lupus erythematosus: report of three cases
Published in Modern Rheumatology Case Reports, 2021
Kento Ichikawa, Yohei Kirino, Yosuke Kunishita, Daiga Kishimoto, Kaoru Takase-Minegishi, Ryusuke Yoshimi, Hideaki Nakajima
A 24-year-old woman presented with facial malar rash and arthralgia. Laboratory data showed high antinuclear antibodies, anti-dsDNA antibody titres, and positive cardiolipin antibodies. Ultrasound screening showed no deep vein thrombosis. No symptoms of lupus nephritis or psychosis were noted. The patient met the 1997 ACR and the 2019 EULAR/ACR classification criteria for SLE and Japanese criteria for Sjögren syndrome (anti-SSA antibody positivity, positive Saxon and Schirmer tests, and presence of superficial keratitis). Her disease activity at the time of HCQ initiation was judged as “moderate” based on EULAR recommendation [9]. Because she did not have organ involvement, she was initiated with daily 200 mg of HCQ. After the induction, her erythema disappeared. anti-dsDNA antibody titre decreased and SLEDAI-2K decreased from 10 to 4. The patient continues to be followed up with HCQ monotherapy.
Reversible cerebral vasoconstriction syndrome triggered by tacrolimus mimicked neuropsychiatric involvement in systemic lupus erythematosus
Published in Modern Rheumatology Case Reports, 2019
Jun Inamo, Jun Kikuchi, Katsuya Suzuki, Yuko Kaneko, Hidekata Yasuoka, Hirokazu Fujiwara, Kunihiro Yamaoka, Tsutomu Takeuchi
In 2017, the malar rash worsened and new discoid lesions appeared on the upper limbs. Titer of anti-dsDNA antibody was elevated up to 66.1 IU/ml after the tapering of PSL to 5 mg/day. Three months after that, she suddenly developed strong headache, nausea and vomiting, and admitted to our department. On physical examination, temperature was 36.9 °C, blood pressure was 131/91 mmHg, pulse rate was 69/min. A malar rash and discoid rash had been present for 3 months. Blood tests showed lymphopenia, hypocomplementemia and elevation of anti-dsDNA antibody titer, suggesting remaining of disease activity (Table 1). We performed magnetic resonance imaging (MRI) to ensure the cause of headache, and then found that the fluid-attenuated inversion recovery (FLAIR) images showed high intensity in the right central sulcus, suggesting subarachnoid haemorrhage. Magnetic resonance angiography (MRA) showed multiple and bilateral, especially in the right middle cerebral artery, constrictive lesions without any aneurysm (Figure 1). In cerebrospinal fluid (CSF), the level of interleukin (IL)-6 elevated to 301 pg/ml. The trough level of tacrolimus was also elevated to 11.6 ng/ml, however, we could not identify the cause of upregulation of the level of tacrolimus, including initiation of new drugs.
Atypical skin manifestations in late-onset lupus: challenging diagnosis
Published in Scandinavian Journal of Rheumatology, 2023
S Méndez-Flores, M Saeb-Lima, G Hernández-Molina
Late-onset lupus is characterized by an onset after 50 years of age. These patients have an insidious course, less severe manifestations, and less accrual damage compared with younger patients (1–5). Skin, articular, and renal involvement are also less common, whereas serositis and Sjögren’s syndrome are more prevalent (2). Regarding skin involvement, some studies reported a lower prevalence of malar rash and photosensitivity (1, 4), while another found a similar frequency (5). On the other hand, the prevalence of discoid lupus, subacute lupus, alopecia, and oral ulcers is similar (4, 6). Also, lower frequencies of vasculitis (1) and, anecdotally, bullous lesions have been reported (7). Herein, we describe two cases of late-onset lupus with atypical skin features of difficult diagnosis.
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