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Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that is difficult to diagnose due to the wide array of signs and symptoms it displays that may be associated to multiple clinical conditions, including perniosis (a rare inflammatory condition), lupus pernio (a manifestation of sarcoidosis), and lupus perniosis (a form of SLE), which can be easily mistaken. Case description: A 29-year-old Colombian mestizo woman with no family history of autoimmune, inflammatory or cutaneous diseases was diagnosed with SLE after ruling out several differential diagnoses. Although the patient presented with features of lupus pernioticus (lupus perniosis), it was established that she had lupus pernio, a type of sarcoidosis. The patient was given the indicated treatment, which led to an improvement in her quality of life. Conclusion: Based on the epidemiology, clinical history and histopathologic findings, it was possible to establish that the patient presented with lupus perniosis and not lupus pernio. In that regard, considering that these three conditions (perniosis, lupus pernio and lupic perniosis) can be easily confused, the present case highlights the importance of a thorough clinical evaluation and precise use of diagnostic terms, because these are three different conditions despite their similar names.
Introducción. El lupus eritematoso sistémico (LES) es una enfermedad autoin-munitaria difícil de diagnosticar debido a la gran variedad de síntomas y signos que ocasionan las múltiples condiciones clínicas que puede provocar, tales como la perniosis (una condición inflamatoria rara), el lupus pernio (una manifestación de la sarcoidosis) y la perniosis lúpica (una forma de LES), las cuales pueden ser fácilmente confundidas. Presentación del caso. Mujer colombiana de 29 años, mestiza y sin antecedente familiar de enfermedades autoinmunes, inflamatorias o cutáneas, quien luego del descarte de varios diagnósticos diferenciales, fue diagnosticada con LES. Aunque la paciente presentó características de lupus perniótico (perniosis lúpica), se estableció que presentaba lupus pernio, un tipo de sarcoidosis. A la paciente se suministró el tratamiento indicado, con lo cual logró una mejoría en sus condiciones de vida. Conclusión. Considerando la epidemiología, la historia clínica y los hallazgos histopatológicos, se puede establecer que la paciente presentó perniosis lúpica y no lupus pernio. En este sentido, teniendo en cuenta que la perniosis, el lupus pernio y la perniosis lúpica pueden confundirse, el presente caso pone de manifiesto la importancia de hacer una evaluación clínica completa y usar los términos diagnósticos más precisos, pues aunque sean similares en nombre, estas son tres condiciones diferentes.
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Chilblain lupus is a rarely manifested variant of chronic lupus. Its appearance can be sporadic or hereditary associated with an autosomal dominant mutation. The diagnosis is clinical and histopathological. The case is presented of a patient with systemic lupus erythematosus presenting with chilblain lupus and nail involvement, despite the use of antimalarials and immunomodulators.
La perniosis lúpica es una variante del lupus crónico que se manifiesta con poca frecuencia, su aparición puede ser esporádica o hereditaria, asociada con una mutación autosómica dominante, en tanto que su diagnóstico es clínico e histopatológico. Se reporta el caso de una mujer con lupus eritematoso sistémico con manifestación de perniosis lúpica y compromiso ungueal, a pesar del uso de antimaláricos e inmunomoduladores.
Subject(s)
Humans , Female , Middle Aged , Skin and Connective Tissue Diseases , Wounds and Injuries , Chilblains , Connective Tissue Diseases , Cold Injury , Frostbite , Lupus Erythematosus, SystemicABSTRACT
Context: The clinical picture of pediatric severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection differs from adults as do the cutaneous manifestations. In this review, we summarize the varied morphological manifestations of SARS-CoV-2 infection in the pediatric population. Evidence acquisition: A comprehensive literature search was conducted (23 September, 2021) across multiple databases (PubMed, EMBASE, MEDLINE and Cochrane) with the relevant keywords. An additional filter of age group between 0-18 years was kept in each of the searches. Results: Chilblains constitute the most common cutaneous manifestation of pediatric coronavirus disease (COVID-19). Other commonly reported manifestations include maculopapular rash, urticaria, erythema multiforme, and papulovesicular eruptions. Majority of children with these manifestations are asymptomatic, highlighting the need to clinically suspect and appropriately manage such patients. A subset of pediatric patients develop severe multisystem involvement termed as multi-system inflammatory syndrome in children (MIS-C) that has varied mucocutaneous manifestations. Conclusion: A wide variety of dermatological manifestation of SARS-CoV-2 infection is reported, and both the pediatrician and dermatologist need to be aware of the same to suspect and diagnose COVID-19 infection in children.
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The clinical, imaging, genetic, therapeutic and prognostic features of a case of pediatric stroke who was finally diagnosed with Aicardi-Goutières syndrome (AGS) in Xi′an International Medical Center Hospital on October 24, 2021 were reported. A 10-year-old boy was admitted to the hospital due to weakness of the right limb for more than 10 hours. The pre-hospital CT showed multiple patchy calcifications in the bilateral frontal lobe and the right parietal lobe cortex-medullary junction. The physical examination on admission had chilblains on the hands, feet and face. National Institutes of Health Stroke Scale Score was 4 points. Brain magnetic resonance imaging showed acute brainstem infarction, no abnormality in magnetic resonance angiography, ultrasound and electrocardiogram of heart and neck vessels were normal, cerebrospinal fluid biochemistry and routine examination were normal, blood routine, biochemistry, coagulation, autoantibody series, thyroid function, tumor markers, human immunodeficiency virus and syphilis examinations were normal. After oral administration of aspirin anti-platelet aggregation and rehabilitation exercises, the muscle strength returned to normal and the patient was discharged. One month later, the result of genetic testing was reported as AGS caused by TREX1 gene mutation, and the mutation site is c.58G>A. AGS is a rare autoimmune hereditary encephalopathy with a large heterogeneity of clinical manifestations. When a hereditary disease was suspected, genetic testing should be done.
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COVID-19 can be accompanied by a variety of cutaneous abnormalities, which mainly include vascular lesions (chilblain-like lesions, livedo reticularis, purpura, ecchymosis, acral cyanosis, gangrene, etc) and inflammatory lesions (diffuse erythema, morbilliform exanthem, acute urticaria, varicella-like exanthem, etc) . Some types of skin lesions may be the first symptom or the only clinical manifestation of COVID-19.
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Objective@#To investigate the correlation between hyperuricemia and non-frozen cold wounds (chilblains) in agricultural workers of a township for prevention measures.@*Methods@#From October 2016 to September 2017, a cross sectional study was applied to 3107 agricultural workers of a township in kaihua county, zhejiang province, of which 371 people with hyperuricemia were observed as the objective group and of which 2 736 people without hyperuricemia as the control group. The data of the occurrence of non-frozen cold wounds (chilblains) in winter among the objective group and the control group were gathered. The descriptive statistics was carried out with the prevalence ratio (PR) of chilblains and 95% confidence intervals of those agricultural workers with hyperuricemia.@*Results@#The prevalence rate was 11.94% and 371 cases with hyperuricemia among 3107 agricultural workers of a township. The total number of cases of non-frozen cold wounds (chilblains) was 195 cases and the prevalence rate was 6.28% with Chilblains among 3 107 agricultural workers of a township. The total of 55 cases with chilblains (14.82%) among the objective group, and 140 cases with chilblains (5.12%) were found among the control group (χ2=52.342, P<0.05) . The objective group had a high prevalence of chilblains. The prevalence ratio (PR) of chilblains was 2.897 (95%CI 2.163-3.881) .@*Conclusion@#Hyperuricemia may be a risk factor for non-frozen cold wounds (chilblains) . Those agricultural workers with hyperuricemia should pay more attention to keep warm and prevent non-frozen cold wounds (chilblains) in winter.
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The human capacity for physiologic adaptation to cold is minimal. A cold environment can be a threat to the skin, leading to a subsequent fall in core body temperature. Many physiologic, behavioral, and environmental factors predispose to the global effects of cold injuries. Physical injuries caused by cold have two forms: systemic forms such as hypothermia and localized forms such as frostbite. Reduced temperature directly damages the tissue, as in frostbite and cold immersion foot. Vasospasm of vessels perfusing the skin induces chilblain, acrocyanosis, and frostbite. The degree of damage caused by cold is related to four factors: temperature, exposure time, wind intensity (temperature sensation), and high altitude.
Subject(s)
Humans , Adaptation, Physiological , Altitude , Body Temperature , Chilblains , Cold Injury , Frostbite , Hypothermia , Immersion Foot , Skin , WindABSTRACT
<p>Chilblains is one indication for the use of an ethical Kampo formulation, i.e. <i>tokishigyakukagoshuyushokyoto</i>. This disease is a very common disorder which occurs in cold circumstances. It has been noted that this disorder correlates with diminished peripheral blood flow. However, the inflammatory mechanism associated with this disorder are still unknown. The author's intention is to speculate on the mechanism behind this disorder from the view point of homeostatic inflammation, as proposed by recent immunology. To wit, chilblains may occur via cytokines which are released from ischemic vessels of the microcirculation and/or cells associated with micro-coagulation in the post-capillary venula.</p>
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En la medicina convencional se denominan acrosíndromes a todas las enfermedades que afectan la microcirculación cutánea; sin embargo es de suma importancia diferenciarlos entre ellos, puesto que aunque comparten características clínicas similares, su causa, pronóstico y tratamiento es diferente. En este artículo se describen las enfermedades más representativas de este grupo de entidades, tales como fenómeno de Raynaud, acrocianosis, livedo reticularis, eritromelalgia, eritema pernio y enfermedad de Buerger; haciendo una descripción detallada de cada una, para que el lector consiga hacer un adecuado diagnóstico diferencial y logre enfocar correctamente los pacientes en la práctica médica.
In conventional medicine, are called acrosyndromes all diseases that affect the skin microcirculation, however it is important to differentiate between them, because although they share similar clinical characteristics; cause, prognosis and treatment is different, and the distinction between them, will achieve impact in the quality of life of patients. Throughout this article, the most representative diseases in this group are described as: Raynaud's phenomenon, acrocyanosis, livedo reticularis, erythromelalgia, erythema pernio and Buerger disease; doing a detailed description of each, emphasizing what makes them unique, thus the reader gets to make an appropriate differential diagnosis and will approach properly the patients with these conditions.
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BACKGROUND: Chilblains are rather common in winter months in our country. OBJECTIVES: Determination of demographical and clinical characteristics of patients presenting with chilblains in Diyarbakir, Turkey. Evaluation of body mass index (BMI) as risk factor for development of chilblains. METHODS: Sixty-nine patients who were diagnosed as chilblains at our outpatient clinics were included in this study. No exclusion criteria were sought. Demographical data including height and weight and answers to questions related to personal history were recorded. A control group consisting of 108 controls were chosen from among patients presenting with complaints of hair loss. Statistical analyses were conducted using SPSS 17.0. RESULTS: Of the 69 patients with chilblains, 45 (65.2%) were females and 24 (34.8%) were males. Mean age (±SD) of the patients was 21±9. 71.2% of the patients complained of itching, 51.6% of pain, 31.3% of burning in the lesions. 61.3% of the patients reported recurrence of the lesions every winter season. Mean BMI of the patients was 20.5±2.9. Mean BMI of the controls was 22.4±3.7 and the difference was statistically significant (p=0.01). CONCLUSIONS: Low BMI may be a risk factor contributing to development of chilblains in persons exposed to cold weather conditions. Apart from external factors, there seems to be a personal tendency to develop lesions, as many patients report recurrences every year. Diagnosis of chilblains requires wariness, as history of cold exposure may not be evident. .
Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Young Adult , Body Mass Index , Chilblains/etiology , Age Factors , Body Height , Body Weight , Case-Control Studies , Regression Analysis , Risk Factors , Seasons , Sex Factors , Socioeconomic Factors , TurkeyABSTRACT
Chilblains are an inflammatory skin condition that develops following exposure to the cold. They consist of pruritic and/or painful erythematous-to-violaceous acral lesions and are the result of an abnormal vascular response to exposure to the cold. This benign condition is common in southern Brazil, particularly in Rio Grande do Sul where climatic conditions, together with cultural and economic aspects, facilitate the development of these sores.
Eritema pérnio é uma condição inflamatória da pele que ocorre após exposição ao frio e se manifesta com lesões eritêmato-violáceas, pruriginosas e dolorosas nas extremidades. Ocorre devido a uma resposta vascular alterada à exposição ao frio. Esta condição benigna é frequente na região sul do Brasil, especialmente no Rio Grande do Sul, onde as características clínicas, associadas a aspectos culturais e econômicos, promovem o desenvolvimento do eritema pérnio.