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11 Possible Causes for Exanthema, Splenomegaly, Systemic Autoinflammation

  • Familial Mediterranean Fever

    Intrinsic apoptosis seemed irrelevant for confining synovial autoinflammation, but regulation through pyroptosis or the adaptive immune system remains possible. arthritis[] Her persistent left elbow effusion resolved and her splenomegaly rapidly disappeared.[] Clinical differential diagnoses encompass erysipelas, erysipelas-like rashes after joint replacements, mercury exanthemas and malignant counter-parts (e.g. erysipelas melanomatosa[]

  • CINCA Syndrome

    […] autoinflammatory conditions characterized by chronic systemic inflammation due to an abnormal regulation of the innate immune system.[] Failure to thrive, mild splenomegaly, and refractory iron-deficiency anemia, without a detectable source of infection, led to no definitive diagnosis, and an extensive investigation[] Nausea, vomiting and abdominal pain with flares, or with elevated intracranial pressure (ICP). [6] Lymphatic: Splenomegaly.[]

  • Erythema Nodosum

    Ahmet Gül, Behçet Disease, Textbook of Autoinflammation, 10.1007/978-3-319-98605-0_35, (647-665), (2019).[] Imaging also revealed splenomegaly and small para-aortic lymph nodes.[] […] skin syndrome, Impetigo, Boil, Carbuncle ) · Strep ( Impetigo ) · Corynebacterium ( Erythrasma ) Viral skin disease Wart · Molluscum contagiosum · Erythema infectiosum · Exanthema[]

  • Vasculitis

    To date, several cases of systemic lupus erythematosus, Aicardi-Goutieres syndrome, familial chilblain lupus, retinal vasculopathy-cerebral leukodystrophy have been reported[] Gabriele Hahn, Pädiatrische Radiologie, Universitätsklinikum Carl Gustav Carus, TU Dresden); (B) Necrotizing skin vasculitis on legs and feet; (C) Splenomegaly, spleen with[] […] 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[]

  • Cryopyrin-Associated Periodic Syndrome

    Hoffman, Autoinflammation: translating mechanism to therapy, Journal of Leukocyte Biology, 90, 1, (37-47), (2011). Keith A. Sikora and Alexei A.[] Inflammation of the thin layer on top of the white part of eye 0100534 Hepatomegaly Enlarged liver 0002240 Progressive sensorineural hearing impairment 0000408 Skin rash 0000988 Splenomegaly[] 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.[]

  • Sweet Syndrome

    French, Autoinflammation behind the curtain, British Journal of Dermatology, 178, 3, (581-582), (2018). Pedro Secchin, Maria K. Gomes, Danielle C.[] Other clinical features can include splenomegaly, storage liver disease, thrombocytopenia, and anemia.[] Pulmonary system – neutrophilic alveolitis, pleural effusions, airway obstruction Liver – hepatitis, hepatomegaly Intestines – neutrophilic inflammation of intestines Spleen – splenomegaly[]

  • Pericarditis

    More recently, there has been evidence that the innate immune system and autoinflammation contribute to acute and recurrent pericarditis. [6, 7] Chronic idiopathic pericarditis[] Splenomegaly Serologic findings include: a. Hypergammaglobulinemia b. High levels of rheumatoid factor c. High levels of antinuclear antibody d.[] […] 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[]

  • Ichthyosis and Male Hypogonadism

    […] autosomal dominant systemic disorder characterized by recurrent blistering skin lesions with a dense inflammatory infiltrate and variable involvement of other tissues, including[] […] 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[] […] deposition in the glomerulus Discoid lupus rash Macroscopic hematuria Loss of eyelashes Decreased serum complement factor I Growth delay Fever Nevus Inguinal hernia Cellulitis Splenomegaly[]

  • Anonychia Congenita Totalis

    […] with infantile enterocolitis (AIFEC) Immunoglobulin G4-related disease Eosinophilic sinusitis Nervous system diseases Neurodegenerative diseases Alzheimer’s disease Lewy[] […] viral infections Abnormal size of pituitary gland Severe B lymphocytopenia Seizures Recurrent hypoglycemia Cataract Muscle weakness Growth delay Hepatomegaly Macrocephaly Splenomegaly[] […] 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[]

  • Early-Onset Sarcoidosis

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

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