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11 Possible Causes for Exanthema, Hepatomegaly, Systemic Autoinflammation

  • CINCA Syndrome

    Some with retinal scarring, corneal haze or vision loss. [6], [26] Cardiopulmonary: Some may have a pericardial effusion, or pericarditis. [1] Abdominal: Some patients have hepatomegaly[autoinflammatory-search.org] […] autoinflammatory conditions characterized by chronic systemic inflammation due to an abnormal regulation of the innate immune system.[ncbi.nlm.nih.gov] Abnormal enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly) and the spleen (splenomegaly) may also occur in some cases.[rarediseases.org]

  • Vasculitis

    In addition to the classical ones, KD may be manifested through a wide variety of symptoms including diarrhoea, hepatomegaly, jaundice, arthritis, myocarditis, pericarditis[elsevier.es] To date, several cases of systemic lupus erythematosus, Aicardi-Goutieres syndrome, familial chilblain lupus, retinal vasculopathy-cerebral leukodystrophy have been reported[ncbi.nlm.nih.gov] […] disease include fever lasting over 5 days, bilateral congestion of the conjunctiva, erythema of the lips, buccal mucosa and the tongue, cervical lymphadenopathy, polymorphous exanthema[symptoma.com]

  • Pericarditis

    He had leg oedema, hepatomegaly, jugular venous distension, and bilateral pleural effusion on chest radiography.[doi.org] More recently, there has been evidence that the innate immune system and autoinflammation contribute to acute and recurrent pericarditis. [6, 7] Chronic idiopathic pericarditis[emedicine.com] […] complex with a key role in innate immunity) lead to overproduction of pro-inflammatory cytokines such as IL-1β, triggering different inflammatory symptoms, such as fever, exanthema[analesdepediatria.org]

  • Erythema Nodosum

    Splenomegaly, hepatomegaly, lymphadenopathy and pleuritis have been rarely seen among EN patients.[lecturio.com] […] skin syndrome, Impetigo, Boil, Carbuncle ) · Strep ( Impetigo ) · Corynebacterium ( Erythrasma ) Viral skin disease Wart · Molluscum contagiosum · Erythema infectiosum · Exanthema[wikidoc.org] Ahmet Gül, Behçet Disease, Textbook of Autoinflammation, 10.1007/978-3-319-98605-0_35, (647-665), (2019).[doi.org]

  • Cryopyrin-Associated Periodic Syndrome

    […] foot [ more ] 0001769 Conjunctivitis Pink eye 0000509 Cranial nerve paralysis 0006824 Episcleritis Inflammation of the thin layer on top of the white part of eye 0100534 Hepatomegaly[rarediseases.info.nih.gov] Hoffman, Autoinflammation: translating mechanism to therapy, Journal of Leukocyte Biology, 90, 1, (37-47), (2011). Keith A. Sikora and Alexei A.[doi.org] Binstadt, Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis, Pediatric Research, 10.1038/pr.2013.187, 75, 1-2, (176-183), (2013). T.[doi.org]

  • Familial Mediterranean Fever

    At age 6 months she was admitted with pericarditis, followed by further admissions with episodic vomiting, lethargy, poor feeding, diarrhea, and hepatomegaly.[bloodjournal.org] Clinical differential diagnoses encompass erysipelas, erysipelas-like rashes after joint replacements, mercury exanthemas and malignant counter-parts (e.g. erysipelas melanomatosa[oatext.com] Intrinsic apoptosis seemed irrelevant for confining synovial autoinflammation, but regulation through pyroptosis or the adaptive immune system remains possible. arthritis[pediatrics.aappublications.org]

  • Sweet Syndrome

    […] myocarditis, aortitis and aortic stenosis, coronary artery occlusion Pulmonary system – neutrophilic alveolitis, pleural effusions, airway obstruction Liver – hepatitis, hepatomegaly[mulicia.pixnet.net] French, Autoinflammation behind the curtain, British Journal of Dermatology, 178, 3, (581-582), (2018). Pedro Secchin, Maria K. Gomes, Danielle C.[doi.org] We describe 4 patients with early-onset recurrent fevers, annular violaceous plaques, persistent violaceous eyelid swelling, low weight and height, lipodystrophy, hepatomegaly[thedoctorsdoctor.com]

  • Ichthyosis and Male Hypogonadism

    Functional respiratory abnormality Elevated serum creatinine Thoracic dysplasia Short iliac bones Hypohidrosis Chronic tubulointerstitial nephritis Scoliosis Flexion contracture Hepatomegaly[mendelian.co] […] acute appendicitis 【急性中毒性表皮剥離(剥脱)】*epidermolysis acuta toxica[L] (略 EAT) 【急性洞炎】*acute sinusitis 【急性脳症】*acute encephalopathy 【急性副鼻腔炎】*acute paranasal sinusitis 【急性発疹】*acute exanthema[medo.jp] […] autosomal dominant systemic disorder characterized by recurrent blistering skin lesions with a dense inflammatory infiltrate and variable involvement of other tissues, including[mendelian.co]

  • Anonychia Congenita Totalis

    ANEMIA, CONGENITAL DYSERYTHROPOIETIC, TYPE Ib; CDAN1B Is also known as cda, type ib Related symptoms: Autosomal recessive inheritance Short stature Ptosis Anemia Hepatomegaly[mendelian.co] […] with infantile enterocolitis (AIFEC) Immunoglobulin G4-related disease Eosinophilic sinusitis Nervous system diseases Neurodegenerative diseases Alzheimer’s disease Lewy[csirnotes.com] […] subitum [sixth disease] B08.20 Exanthema subitum [sixth disease], unspecified B08.21 Exanthema subitum [sixth disease] due to human herpesvirus 6 B08.22 Exanthema subitum[eicd10.com]

  • Early-Onset Sarcoidosis

    Some cases with lung involvement. [34] [35] Abdominal: Hepatomegaly, splenomegaly or hepatosplenomegaly.[autoinflammatory-search.org] The underlying mechanisms of Blau syndrome leading to autoinflammation are still unclear, and there is currently no effective specific treatment for Blau syndrome.[readbyqxmd.com] The initial symptoms comprise a typical exanthema, with tiny round lesions that vary in colour from pale pink to tan or intense erythema.[printo.it]

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