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CUTANEOUS MANIFESTATIONS OF SYSTEMIC DISEASES: A RETROSPECTIVE STUDY ANALYZED STUDY

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Cutaneous lupus erythematosus (CLE) is a chronic inflammatory skin disease characterized by a diverse cadre of clinical presentations. CLE commonly occurs in patients with systemic lupus erythematosus (SLE), and CLE can also develop in the absence of systemic disease. Although CLE is a complex and heterogeneous disease, several studies have identified common signaling pathways, including those of type I interferons (IFNs), that play a key role in driving cutaneous inflammation across all CLE subsets. However, discriminating factors that drive different phenotypes of skin lesions remain to be determined. Thus, we sought to understand the skin-associated cellular and transcriptional differences in CLE subsets and how the different types of cutaneous inflammation relate to the presence of systemic lupus disease. In this study, we utilized two distinct cohorts comprising a total of 150 CLE lesional biopsies to compare discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus (ACLE) in patients with and without associated SLE. Using an unbiased approach, we demonstrated a CLE subtype-dependent gradient of B cell enrichment in the skin, with DLE lesions harboring a more dominant skin B cell transcriptional signature and enrichment of B cells on immunostaining compared to ACLE and SCLE. Additionally, we observed a significant increase in B cell signatures in the lesional skin from patients with isolated CLE compared with similar lesions from patients with systemic lupus. This trend was driven primarily by differences in the DLE subgroup. Our work thus shows that skin-associated B cell responses distinguish CLE subtypes in patients with and without associated SLE, suggesting that B cell function in skin may be an important link between cutaneous lupus and systemic disease activity.
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The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors' intention is that, by using direct and easily accessible language, the authors aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.
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Lupus, Latin for “wolf,” is a term used to describe many dermatologic conditions, some of which are related to underlying systemic lupus erythematosus, while others are distinct disease processes. Cutaneous lupus erythematosus includes a wide array of visible skin manifestations and can progress to systemic lupus erythematosus in some cases. Cutaneous lupus can be subdivided into three main categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Physical exam, laboratory studies, and histopathology enable differentiation of cutaneous lupus subtypes. This differentiation is paramount as the subtype of cutaneous lupus informs upon treatment, disease monitoring, and prognostication. This review outlines the different cutaneous manifestations of lupus erythematosus and provides an update on both topical and systemic treatment options for these patients. Other conditions that utilize the term “lupus” but are not cutaneous lupus erythematosus are also discussed.
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Inflammatory bowel diseases (IBDs) may be associated with extra-intestinal manifestations. Among these, mucocutaneous manifestations are relatively frequent, often difficult to diagnose and treat, and may complicate the course of the underlying disease. In the present review, a summary of the most relevant literature on the dermatologic manifestations occurring in patients with inflammatory bowel diseases has been reviewed. The following dermatological manifestations associated with IBDs have been identified: (i) specific manifestations with the same histological features of the underlying IBD (occurring only in Crohn’s disease); (ii) cutaneous disorders associated with IBDs (such as aphthous stomatitis, erythema nodosum, psoriasis, epidermolysis bullosa acquisita); (iii) reactive mucocutaneous manifestations of IBDs (such as pyoderma gangrenosum, Sweet’s syndrome, bowel-associated dermatosis-arthritis syndrome, aseptic abscess ulcers, pyodermatitis–pyostomatitis vegetans, etc.); (iv) mucocutaneous conditions secondary to treatment (including injection site reactions, infusion reactions, paradoxical reactions, eczematous and psoriasis-like reactions, cutaneous infections, and cutaneous malignancies); (v) manifestations due to nutritional malabsorption (such as stomatitis, glossitis, angular cheilitis, pellagra, scurvy, purpura, acrodermatitis enteropathica, phrynoderma, seborrheic-type dermatitis, hair and nail abnormalities). An accurate dermatological examination is essential in all IBD patients, especially in candidates to biologic therapies, in whom drug-induced cutaneous reactions may assume marked clinical relevance.
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Background: The coronavirus disease 2019 (COVID-19) pandemic has affected the whole world for a short span of time. As it is a novel virus, its manifestations have been slowly revealed. Cutaneous manifestations of COVID-19 have gradually been reported from different regions of the world. However, their significance in diagnosis, as well as predicting prognosis, remains to be established. Objective: This review describes the cutaneous manifestations of COVID-19 patients from diverse regions with the aim of highlighting any important associations. Methods: A literature search was conducted using "PubMed" for original articles, case series, and case reports using the search terms "cutaneous manifestations" and "skin manifestations" in combination with "COVID-19" published up to 31 May. Results: Nine original research articles and 35 case series or case reports were identified, including 458 confirmed COVID-19 cases. The ratio of male to female patients was 0.94, and the patients' age ranged from 2 months to 84 years. In 10% of cases, skin lesions appeared before systemic manifestations. The most common cutaneous manifestation was macular/maculo-papular rash (42.5%), followed by acute urticaria (17.9%), vesicular rash (15.3%), pseudo-chilblain or acral purpuric lesions (15.1), and livedo-reticularis (4.4%). The majority of reported cases were located in Spain, Italy, and France. These manifestations do not seem to be sex-, age-, or country-specific. Conclusion: It is necessary to conduct worldwide registries and prospective studies to assess the true incidence of cutaneous manifestations of COVID-19 and to streamline their categorization based on their pathogenesis. There appears to be no specific manifestation at present, but a high index of suspicion can help in the diagnosis of patients presenting with cutaneous lesions as the first manifestation.
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Pancreatic pathology, comprising acute and chronic pancreatitis, autoimmune pancreatitis and pancreatic neoplasms, primarily presents with gastrointestinal symptoms and signs; however, it is well recognized that it can also associate a wide range of extra-digestive features. Among these systemic manifestations, cutaneous involvement plays an important role both as a diagnostic clue for the pancreatic disease itself and serving as a prognostic factor for the severity of the condition. Recognition of these cutaneous signs is, however, far from being satisfactory, all the more as some of them are relatively rare. In the current review, we discuss skin involvement in pancreatic diseases, referring to pancreatic panniculitis, cutaneous hemorrhagic manifestations, skin metastasis, acanthosis nigricans, livedo reticularis, necrolytic migratory erythema and cutaneous fistula. We highlight the clinical characteristics, treatment and prognostic value of these lesions. Better awareness among medical specialties other than dermatology is needed for detection of the skin clues associated with pancreatic pathology.
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The majority of patients with systemic lupus erythematosus (SLE) have cutaneous manifestations at some point in their disease. The skin findings in lupus erythematosus are classified as lupus-specific or lupus-nonspecific based on histopathologic findings. Lupus-specific skin diseases include chronic cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and acute cutaneous lupus erythematosus. There are subsets of skin lesions within each group and the likelihood of associated SLE varies amongst them. Lupus nonspecific lesions are more common in patients with SLE and tend to coincide with active systemic disease. Lupus nonspecific lesions may be seen as a feature of another disease process, including other connective tissue diseases. It is important for the rheumatologist to be familiar with the spectrum of cutaneous disease in lupus erythematosus to help prognosticate the likelihood of systemic disease and ensure patients receive timely dermatologic care with the goal of controlling disease activity to prevent damage.
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The cutaneous manifestations of COVID‐19 patients have been increasingly reported, but not summarized, and the potential mechanisms remain to be investigated. Herein, we performed a comprehensive review of literatures (from inception to May 30, 2020) using PubMed, CNKI, medRxiv, and bioRxiv with the terms “((novel coronavirus) OR (2019 novel coronavirus) OR (2019‐nCoV) OR (Coronavirus disease 2019) OR (COVID‐19) OR (SARS‐CoV‐2)) AND ((Dermatology) OR (skin) OR (rash) OR (cutaneous))” and “((ACE2) OR (Angiotensin‐converting enzyme)) AND ((skin) OR (epidermis) OR (dermis))”. Totally, 44 articles met the inclusion criteria. A total of 507 patients with cutaneous manifestations were summarized, and 96.25% patients were from Europe. The average age of the patients was 49.03 (range: 5‐91) with a female ratio of 60.44%. The skin lesions were polymorphic, and erythema, chilblain‐like and urticarial lesions were most common, occurring on an average of 9.92 days (range: 1‐30) after the onset of systemic symptoms. The receptor of SARS‐CoV‐2, ACE2, was found to be expressed on skin, mainly on keratinocytes. Our review systematically presented the clinical characteristics of 507 patients and showed that skin might be the potential target of the infection according to ACE2 expression. More work should be done to better understand the underlying pathogenesis.
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Given the visibility of cutaneous findings, skin manifestations are often a presenting symptom of underlying systemic disease, including chronic liver disease. Many cutaneous signs and symptoms that correlate with chronic liver disease are common physical examination findings in patients with no history of liver disease. It is nonetheless important to be aware that these cutaneous findings may be an indication of underlying liver disease and often occur in the setting of such hepatic dysfunction. This article covers general cutaneous signs that may correlate with various liver diseases and describes specific cutaneous signs as they relate to more specific liver diseases.
Cutaneous Manifestations of Systemic Diseases: A Retrospective Study at PIMS Hospital
  • K M A Azeem
Azeem, K. M. A. et al. (2022). Cutaneous Manifestations of Systemic Diseases: A Retrospective Study at PIMS Hospital, Islamabad. Cureus, 14(3), e4002. https://doi.org/10.7759/cureus.4002
The Skin as a Mirror of Systemic Disease
  • J Smith
Smith, J. (2010). The Skin as a Mirror of Systemic Disease. Journal of the American Academy of Dermatology, 62(3), 489-500. https://doi.org/10.1016/j.jaad.2009.10.077
Dermatological Evaluation in Systemic Diseases
Dermatological Society of Pakistan. (2015). Dermatological Evaluation in Systemic Diseases. Journal of Dermatology and Clinical Research, 3(1), 1024. https://doi.org/10.15406/jdcr.2015.03.00102