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Sjögren syndrome and mixed connective tissue disease
Published in Biju Vasudevan, Rajesh Verma, 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.
Photoexacerbated Dermatoses
Published in Henry W. Lim, Nicholas A. Soter, Clinical Photomedicine, 2018
Photosensitivity, defined as a skin rash as a result of unusual reaction to sunlight, documented by history or observed by physician, is included as a criterion distinct from malar rash and discoid lupus in the 1982 American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE) (1). Between 15 and 19% of black patients with SLE are photosensitive compared to 32–71% of white patients with SLE (2–4) (Fig. 1, see color plate). The presence of photosensitivity correlates with the incidence of antibodies to the cytoplasmic antigen Ro (SS-A) (4,5), with photosensitivity being present in up to 90% of Ro-positive patients (5). In one series, Ro-positivity was determined to be a greater predictor of photosensitivity in white patients. In white patients, 87% of Ro-positive and 54% of Ro-negative patients were photosensitive, while in black patients, 4% of Ro-positive and 55% of Ro-negative individuals were photosensitive. These findings led the authors to suggest that Ro-positive black patients may have a blocking factor that protects them against photosensitivity (4).
Connective tissue diseases
Published in Aimilios Lallas, Enzo Errichetti, Dimitrios Ioannides, Dermoscopy in General Dermatology, 2018
Enzo Errichetti, Aimilios Lallas
ACLE is almost invariably associated with systemic manifestations (systemic LE).1,2 Malar rash or butterfly erythema is the most common presentation and consists of an erythema (with or without edema) over the malar areas and bridge of the nose with a usual nasolabial folds sparing (Figure 4.1A).1,2 A macular or diffuse erythematous rash on sun-exposed areas, erythematous papules, or macules on the dorsal aspects of the hands, typically sparing the knuckles, and alopecia (Figure 4.2A) may also be seen.1,2
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.
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.
A Long-Term Follow-up of Retinal Vasculitis – Do They Develop Systemic Disease?
Published in Ocular Immunology and Inflammation, 2020
Ankush Kawali, Bharathi Bavaharan, Srinivasan Sanjay, Ashwin Mohan, Padmamalini Mahendradas, Bhujang Shetty
Three cases developed nonspecific joint pain during follow-up, one case developed seizure (after completion of ATT and while on Azathioprine after 1.5 years). Two cases developed psoriasis; both the patients were initially treated as PTV. One of them developed the skin lesions 2 years after discontinuation of steroids and had occlusive vasculitis at presentation while another developed psoriatic skin lesions after 8 months of presentation and had exudative vasculitis. In both the cases no relapse of vasculitis was seen after completion of ATT as well as after the diagnosis of psoriasis. One case was diagnosed as Systemic Lupus Erythematosus (SLE) after 5 years of presentation when the patient developed a malar rash. This patient also had occlusive vasculitis and was treated with ATT and steroids initially. Another case developed ulcerative colitis, 16 months after presentation, while on AZA. In total only 4 of 76 cases (5.26%) developed a systemic disease which can be attributed to their retinal vasculitis (Table 1)