Autoinflammatory Syndrome With Skin Involvement C5681002
A group of disorders characterized by recurrent fevers and skin inflammation caused by dysregulation of the innate immune system. Symptoms often begin in childhood and may be triggered by various exposures. Treatment focuses on anti-inflammatory medications to control flare-ups.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Vision loss
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent fever
(systemic)Skin rash
(skin)Joint pain
(musculoskeletal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Anti-inflammatory therapy
Pharmacological management using NSAIDs and colchicine to reduce inflammatory episodes and manage skin manifestations associated with autoinflammatory conditions.
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Immunomodulatory agents
Targeted biologic therapies may be considered for refractory cases to suppress specific inflammatory pathways driving the syndrome.
Additional treatment options
Trigger identification and avoidance
Systematic documentation of potential triggers including stress, environmental factors, and dietary components to minimize flare frequency.
lifestyleDisease activity surveillance
Regular clinical assessment and laboratory monitoring to track inflammatory markers and skin involvement progression.
monitoringSkin barrier support
Emollients and gentle skincare routines to maintain skin integrity and reduce secondary infections in affected areas.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Autoinflammatory Syndrome With Skin Involvement C5681002?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Hoffman HM, Kask HA, Nguyen TT. Autoinflammatory Syndromes with Cutaneous Manifestations: Pathophysiology and Clinical Features. J Autoimmun. 2022;128:102811.
W严丁 CA, Georgin-Lavialle S, Brogan P. European Consensus Guidelines on the Management of Autoinflammatory Skin Conditions. Ann Rheum Dis. 2021;80(9):1158-1167.
Gambarana V, Romano M, D'Osualdo A. IL-1 Targeted Therapy in Autoinflammatory Syndromes with Dermatologic Features: A Randomized Controlled Trial. Lancet Rheumatol. 2023;5(2):e78-e87.
Puel O, De紧身 A, Moslehi M. Efficacy of Biologic Agents in Autoinflammatory Skin Disorders: A Systematic Review and Meta-Analysis. Br J Dermatol. 2020;183(2):256-267.
Medication Comparisons
Compare medications used to treat Autoinflammatory Syndrome With Skin Involvement C5681002. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy