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Connective Tissue Disorders
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Laura Atzori, Caterina Ferreli, Franco Rongioletti
Overview: Lupus erythematosus is a complex autoimmune systemic disease with variable clinical features. Skin involvement is common in systemic lupus erythematosus (SLE) and is divided into specific skin lesions with typical histopathology and non-specific skin manifestations (Table 12.1).
Prednicarbate
Published in Anton C. de Groot, Monographs in Contact Allergy, 2021
A 53-year-old atopic woman had a history of dyshidrotic hand eczema and self-limited outbreaks of itchy dermatitis in her face and neck. She presented with itchy, confluent red macules and papules that had evolved into sharply demarcated erythematous plaques localized on her forehead and neck after having applied prednicarbate cream 3 days previously. The clinical diagnosis was lupus erythematosus. A biopsy taken from a lesion on the forehead showed hydropic degeneration of the basal layer, edema, and a lymphocytic infiltrate around the vessels and hair follicles. Immunofluorescence showed IgM and C3 at the dermo-epidermal junction. ANA, anti-ds-DNA and complement were normal or negative. Acute signs disappeared in a few weeks after avoiding the cream. Patch tests were positive to budesonide, prednicarbate cream (ROAT also positive), and prednicarbate 1% pet. and 1% alcohol. The patient was diagnosed with allergic contact dermatitis to prednicarbate, presenting with the clinical and histopathological features of lupus erythematosus (4).
Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Systemic lupus erythematosus causes glomerulonephritis, musculoskeletal symptoms and a rash but it is usually a characteristic butterfly facial rash. Intussusception is not a feature, although there are commonly other gastrointestinal manifestations.
Combination strategies for lupus nephritis: facts and controversies
Published in Expert Review of Clinical Immunology, 2023
Renal involvement is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). The age and sex adjusted standardized mortality ratio (SMR) of lupus nephritis (LN), compared to those without renal disease, was estimated to be 2.23 [1]. The SMR went up to 3.59 and 9.20, respectively, in those with renal damage and end-stage renal disease (ESRD). Despite the treatment advances of SLE and chronic kidney disease (CKD) in the past few decades, ESRD still develops in up to 30% of patients after 10 years of SLE onset [2]. A meta-analysis of 102 publications showed that the 10-year ESRD rate of LN improved from the 1970s to the mid-1990s when it plateaued at around 17% [3]. On the other hand, LN is associated with impairment of the health-related quality of life (HRQoL) [4]. A multinational study of a SLE-specific HRQoL questionnaire (LupusPRO) showed that LN patients had significantly lower scores in the medication and procreation domains than non-renal SLE patients, even after adjustment for age, sex, ethnicity, and country of residence [5].
Retinal findings in glomerulonephritis
Published in Clinical and Experimental Optometry, 2022
Heather G Mack, Deborah J Colville, Phillip Harraka, Judith Anne Savige, Alessandro Invernizzi, Samantha Fraser-Bell
Multiple mechanisms of complement activation, and other pathological mechanisms, are involved in retinal involvement in forms of glomerulonephritis, as exemplified by individuals with systemic lupus erythematosus.95 Systemic lupus erythematosus is a chronic autoimmune tissue disorder, affecting multiple tissue systems, often with a relapsing and remitting course. Lupus nephritis is a severe subtype of systemic lupus erythematosus characterised by activation of multiple complement pathways and presence of autoantibodies to complement components C1q and C3b. Diverse retinal findings (Table 1) include: haemorrhages and nerve fibre layer infarcts in up to 30% of individuals; retinal vasculitis secondary to immune-complex deposition; retinal arteriolar and venular occlusion due to fibrinoid necrosis of vessel walls; central retinal vein occlusion-like picture due to antiphospholipid antibody-related prothrombotic state, and Purtscher-like retinopathy attributed to microemboli. Serous retinal detachment has been frequently reported.96 Choroidopathy,78,96,97 with thickening of the choroid and angle closure glaucoma98 has been reported.
The Overlap between Genetic Susceptibility to COVID-19 and Skin Diseases
Published in Immunological Investigations, 2022
Navid Jabalameli, Fateme Rajabi, Alireza Firooz, Nima Rezaei
The 3p21.31 locus is also familiar with dermatologists as it contains the TREX gene (Hur et al. 2008; Lee-Kirsch et al. 2007). While on genetic and molecular levels not much is known about the interaction of these genes with SARS-CoV-2, there is a clinical point about TREX1 that cannot be ignored. A heterozygous missense mutation (D18N) in the TREX1 gene causes familial chilblain lupus, a condition fairly similar in both clinical and pathological presentations to the notorious COVID toe (Kolivras et al. 2020; Lee-Kirsch et al. 2007, 2006). Polymorphisms of this gene also contribute to the development of systemic lupus erythematosus (Namjou et al. 2011). The TREX1 gene encodes an enzyme involved in granzyme-A mediated apoptosis by degrading DNA. TREX1 also degrades viral nucleic acids (both DNA and single-stranded RNA), which allows viruses such as HIV to remain unrecognized by intracellular sensing elements and escape the interferon-mediated antiviral response (Huang et al. 2018; Stetson et al. 2008; Yan et al. 2010; Yuan et al. 2015). The TREX1 mutation significantly reduces its exonucleolytic activity which results in the accumulation of nucleic acids that can stimulate type-I interferon responses and trigger autoimmunity (Fiehn 2017; Lee-Kirsch et al. 2007; Stetson et al. 2008). Based on the above-mentioned mechanisms, an alteration in TREX1 activity, perhaps due to a promoter/enhancer modulation, may be responsible for the coincidence of both viral infection and an autoimmune phenomenon such as chilblain (Figure 1).