Edit concept Question Editor Create issue ticket

Wells Syndrome

Eosinophilic Cellulitis


Presentation

  • Other reported clinical presentations include papular and nodular eruptions. It may be recurrent, and preceded at a variable time by a pruritic papular eruption.[ncbi.nlm.nih.gov]
Fever
  • Drug Indication Risks Benefits NSAIDS Arthritis; pain, fever Adverse reactions Reduce fever, pain, arthritis Corticosteroids Fever, arthritis,serositis, urticaria, vasculitis Adverse reactions Relieve fever, rash, arthritis, serositis Infliximab Chronic[clinicaladvisor.com]
  • Muckle-Wells Syndrome (MWS) is a rare type of genetic disease that is characterized by occasional occurrences of skin rash, fever and joint pain as well as various other problems.[hxbenefit.com]
  • Besides the cutaneous lesions, patients can experience systemic manifestations like fever, malaise, arthralgia and peripheral blood eosinophilia.[ncbi.nlm.nih.gov]
  • Periodic Fevers Moderate, low-grade fevers are often present, and add to the frequent suffering. Fevers start in early infancy.[autoinflammatory.org]
  • These findings promote awareness of these hereditary periodic fever syndromes as a cause for recurrent fevers from childhood in the Indian population.[jpgmonline.com]
Pseudotumor
  • BACKGROUND: We report a case of a 33-year-old man who presented with immunoglobulin (Ig)G4-related disease (IgG4-RD) forming a pseudotumor in the left paratesticular region during oral administration of corticosteroid for Wells syndrome, which involves[ncbi.nlm.nih.gov]
Pertussis
  • A 4-year-old boy presented with erythematous vesicular plaques, ulceration, edema, and pruritus on the left foot and ankle 10 days after receiving the tetanus, diphtheria, pertussis, and polio; measles, mumps, rubella, and varicella; and hepatitis A/B[ncbi.nlm.nih.gov]
Subcutaneous Mass
  • Author information 1 University of Southern California, Los Angeles, USA. drafsahi@yahoo.com Abstract Wells syndrome, or eosinophilic cellulitis, is a rare disorder characterized by erythematous plaques evolving into dermal or subcutaneous masses.[ncbi.nlm.nih.gov]
Annular Skin Lesion
  • BACKGROUND/OBJECTIVE: Eosinophilic annular erythema (EAE) was proposed to describe annular skin lesions associated with tissue eosinophilia, however, its relation to Well's syndrome (WS) remains a source of controversy.[ncbi.nlm.nih.gov]
Scrotal Mass
  • CASE PRESENTATION: The patient was introduced to our institution from a private hospital with a 3-month history of asymptomatic left scrotal mass. A 5-cm diameter nodule was palpable in the left scrotum.[ncbi.nlm.nih.gov]

Workup

  • It is also important to perform a malignancy workup for any patient with a diagnosis of Wells syndrome that develops B-symptoms such as fevers, night sweats, weight loss, pruritus and decreased appetitie or lethargy, as this may signal an associated lymphoma[dermatologyadvisor.com]
  • Routine laboratory workup revealed a C-reactive protein level of 122 mg/l (normal 4 ). The patient was discharged and was instructed to continue the combined antihistamine therapy to prevent recurrences.[karger.com]
Herpes Simplex Virus Type 2
  • We report the repeated association of eosinophilic cellulitis with herpes simplex virus type 2 infections.[ncbi.nlm.nih.gov]
Toxocara Canis
  • The differential diagnosis of Wells' syndrome is broad and includes infections, such as bacterial cellulitis, Toxocara canis, erythema chronicum migrans, arthropod bites and hypereosinophilc syndrome, chronic idiopathic urticaria and Churg-Strauss syndrome[histopathology-india.net]

Treatment

  • The objective of this study was to review the so far reported treatment options for Wells syndrome in a systematic manner.[ncbi.nlm.nih.gov]
  • Antihistamines, nonsedating as well as sedating, are also first-line treatment.[dermatologyadvisor.com]

Prognosis

  • Due to the good prognosis and tendency to resolve, systemic treatment should be limited to cases resistant to local therapy or with widespread lesions. 2016 European Academy of Dermatology and Venereology.[ncbi.nlm.nih.gov]
  • These include: Abdominal pain Peritonitis Urticaria AA amyloidosis Sensorineural hearing loss Muckle Wells Syndrome Prognosis The prognosis of MWS depends on the possible outcomes of its symptoms.[hxbenefit.com]
  • Muckle-Wells Syndrome synonyms 1 answer Muckle-Wells Syndrome prognosis What is Muckle-Wells Syndrome Which advice would you give to someone who has just been diagnosed with Muckle-Wells Syndrome? 1 answer[diseasemaps.org]
  • Canakinumab, a monoclonal antibody against interleukin-1β Prognosis [ edit ] The chronic inflammation present in MWS over time can lead to deafness.[en.wikipedia.org]
  • An inappropriate hypersensitivity reaction is often postulated. [1] It usually runs a chronic recurrent clinical course with a good prognosis. Clinical features are varied, making diagnosis difficult.[ijpd.in]

Etiology

  • Wells syndrome is a rare disorder of unknown etiology. Precipitants include insect bites, infections, medications, malignancies, and vaccinations. Possible mechanisms include hypersensitivity reactions to antigens.[ncbi.nlm.nih.gov]

Epidemiology

  • […] eosinophilic cellulitis Essential features Idiopathic condition typically characterized by recurrent pruritic to painful plaques that often clinically resemble cellulitis Histopathology characterized by a prominent eosinophilic infiltrate with "flame figures" Epidemiology[pathologyoutlines.com]
  • (A review of the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, and treatment of Wells syndrome.) Wolverton, SE. Comprehensive dermatologic drug therapy. Saunders Elsevier. 2007. pp. 247.[dermatologyadvisor.com]
  • Cuisset L, Jeru I, Dumont B, Fabre A, Cochet E, Le Bozec J et al (2011) Mutations in the autoinflammatory cryopyrin-associated periodic syndrome gene: epidemiological study and lessons from eight years of genetic analysis in France.[link.springer.com]
  • EPIDEMIOLOGY MWS is a rare disorder. Although it has been reported in many regions of the world, the exact prevalence of this disorder is not known properly. MWS is a autosomal dominant pathology that has variable age of onset.[flipper.diff.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology of Muckle Wells Syndrome Protein cryopyrin is partly responsible for the body’s response to damage or infection leading to release of interleukin 1[Produced by an immune cell known as a macrophage] to produce symptoms of inflammation such[boneandspine.com]
  • Pathophysiology The pathophysiology of Wells syndrome remains unclear. It has been postulated that there may be an error in the eosinophil homing mechanism.[dermatologyadvisor.com]
  • "Clinical categories of exaggerated skin reactions to mosquito bites and their pathophysiology". Journal of Dermatological Science. 82 (3): 145–52. doi : 10.1016/j.jdermsci.2016.04.010. PMID 27177994. External links [ edit ] DermNet NZ entry[en.wikipedia.org]
  • Clinical categories of exaggerated skin reactions to mosquito bites and their pathophysiology. J Dermatol Sci. 2016 Jun. 82 (3):145-52. [Medline]. Plotz SG, Abeck D, Behrendt H, Simon HU, Ring J. [Eosinophilic cellulitis (Wells syndrome)].[emedicine.medscape.com]

Prevention

  • Patients on prolonged bedrest should be given heparin to prevent deep venous thrombosis as well as stool softeners to prevent constipation.[medical-dictionary.thefreedictionary.com]
  • In addition, our cases suggest that combination therapy with levocetirizine and hydroxyzine may be successfully used as corticosteroid-sparing treatment or to prevent relapse after the discontinuation of corticosteroid treatment. 2015 S.[karger.com]
  • When this has been accomplished, the body stops (inhibits) the inflammatory response to prevent damage to its own cells and tissues.[flipper.diff.org]
  • It is too soon to tell if early medication treatment for MWS or NOMID/CINCA will prevent or decrease the development of hearing loss, but there is great hope that it will make a significant difference.[autoinflammatory.org]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!