Later on, the clinical presentation changed to erythematous plaques, partially with cockade-like aspects. [ncbi.nlm.nih.gov]

Case Report A 17-month-old child presented with a 2-month recurrent cutaneous rash on all 4 extremities. [mdedge.com]

• 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]

  These findings promote awareness of these hereditary periodic fever syndromes as a cause for recurrent fevers from childhood in the Indian population. [jpgmonline.com]

  MWS (first described in 1962)2 belongs to a group of hereditary periodic fever syndromes. These syndromes are characterised by intermittent attacks of fever. Four of these syndromes have been described. [nature.com]

• Pneumonia

  Rinsho Hifuka Volume 57, Issue 2 （February 2003） 臨床皮膚科 57巻2号 （2003年2月） Japanese English 症例報告 好酸球性肺炎を合併したWells症候群の1例 A Case of Wells' syndrome with eosinophilic pneumonia 宮本 由香里 1, 大塚 俊 1, 山崎 雙次 1 Yukari MIYAMOTO 1, Shun OHTSUKA 1, Soji YAMAZAKI 1 1 Depertment [webview.isho.jp]

  Clinicopathological Correlation in a Series of 13 Patients. 61 Wilk M...Zelger B 31436577 2020 23 Wells' syndrome related to Mycoplasma pneumoniae in a 5-year-old boy. 61 Kim SH...Kim HY 32304128 2020 24 Off-Label studies on anakinra in dermatology: a [malacards.org]

  （UIP）） 肺好酸球性肉芽腫（Pulmonary eosinophilic granuloma） ニューモシスチス肺炎（Pneumocystis jirovecii pneumonia） 逆流性食道炎（Reflux esophagitis） 食道乳頭腫（Squamous papilloma of the esophagus） 胃のMALTリンパ腫（Gastric MALT lymphoma） 胃腺癌（Gastric adenocarcinoma） 胃癌（腺癌）（Adenocarcinoma of [kinpodo-pub.co.jp]

• Dermatitis

  It has been associated with various skin lesions but predominantly with dermatitis herpetiformis. [ncbi.nlm.nih.gov]

  Although contact dermatitis and fungal infection cannot be excluded in this case, the lack of pruritus and grossly visible scaling argue against allergic contact dermatitis. [jcadonline.com]

• Insect Bite

  We recommend meticulous investigation of patients diagnosed with Wells' syndrome manifesting as an insect-bite-like eruption. [ncbi.nlm.nih.gov]

  She denied asthma or atopy, insect bite, or new medications. Skin biopsy (Figure 2) revealed perivascular & interstitial infiltrate composed of lymphocytes, histiocytes, neutrophils and eosinophils. [bloodjournal.org]

  The "flame figures" can also be found in diverse other diseases including insect bite reactions, dermatophyte id reactions, pemphigoid and Churg Strauss syndrome. [1] The pathogenesis of Wells' syndrome is unknown. [ijdvl.com]

• Erythema

  Abstract Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and wheals to an unusual presentation of vesicles and bullae. [ncbi.nlm.nih.gov]

  […] non-infectious cellulitis to know: Wells' syndrome]. 61 Alvarez Martinez D...Laffitte E 33793097 2021 4 Terbinafine-induced DRESS syndrome mimicking eosinophilic cellulitis. 61 Rajagopal Srinivasan V...Vedant D 32780900 2021 5 Case of Muckle-Wells syndrome with erythema [malacards.org]

  Clinical description The lesions are usually filled with fluid, tender and progress to form hardened plaques of edema and erythema. They usually resolve without scarring. [orpha.net]

• Papule

  Here, we report the case of a 46-year-old man who initially presented with excoriated papules that were histologically interpreted as consistent with "arthropod reactions." [ncbi.nlm.nih.gov]

  Abstract Introduction Wells’ syndrome, also known as eosinophilic cellulitis, is a rare inflammatory dermatosis that mostly affects adults and manifests as recurrent erythematous papules, vesicles, and nodules. [journal.pds.org.ph]

  A 39-year-old female presented with a three-year history of diffusely located pruritic papules. After a negative lab and infectious workup, a punch biopsy showed dense eosinophilic infiltrate in the dermis as well as “flame figure” formation. [jofskin.org]

  Here, we report the case of a 46-year-old man who initially presented with excoriated papules that were histologically interpreted as consistent with “arthropod reactions.” [journals.lww.com]

• Blister

  Blistering, generalized Blisters [ more ] 0008066 Edema Fluid retention Water retention [ more ] 0000969 Eosinophilia High blood eosinophil count 0001880 Skin vesicle Blister 0200037 5%-29% of people have these symptoms Arthralgia Joint pain 0002829 [rarediseases.info.nih.gov]

  Within few days, a painful red, infiltrated, blistering plaque developed at the site of the sting. Treatment with oral antibiotics was ineffective. The patient was then subjected to a skin biopay. [ijdvl.com]

  Alergol 2014;31:322 ) and does not improve with antibiotics ( J Clin Aesthet Dermatol 2011;4:55 ) Erythematous papules, plaques or nodules that may be painful or pruritic ( Postepy Dermatol Alergol 2014;31:322 ) Sometimes annular configuration, can blister [pathologyoutlines.com]

  You may also notice small lumps or blisters developing on your skin. Other symptoms include tiredness and fevers. There are no known problems that occur with Well’s Syndrome. [dermcoll.edu.au]

• Burning Sensation

  Symptoms usually begin with an itchy or burning sensation in one or more areas of skin, followed by redness and swelling. These areas are often painful. Sometimes, several new blisters or nodules are the first sign of the condition. [my.clevelandclinic.org]

  There is sudden onset of itching and burning sensation. The affected patients usually present with violaceous plaques over a 2- to 3-day period. The plaques resolve spontaneously without scarring over 2–8 weeks. [histopathology-india.net]

  Pruritus, pain, and a burning sensation can be present before or during the presence of the lesions; however, systemic symptoms (malaise, fever) are rare.9 Peripheral eosinophilia is detected in approximately half of the patients.1,2 After a period of [mdedge.com]

  CASE REPORT A 32-year-old female presented with an erythematous, edematous plaque on the flexor aspect of left forearm of 10 days duration, associated with a prodrome of malaise followed by itching and burning sensation at the site [Figure 1]. [journals.lww.com]

• Neurologic Manifestation

  Kucharczyk M...Krecisz B 31232056 2019 47 The NLRP3 p.A441V Mutation in NLRP3-AID Pathogenesis: Functional Consequences, Phenotype-Genotype Correlations and Evidence for a Recurrent Mutational Event. 61 Awad F...Amselem S 31777803 2019 48 Spectrum of the neurologic [malacards.org]

  Expanding spectrum of neurologic manifestations in patients with NLRP3 low-penetrance mutations. Neurol Neuroimmunol Neuroinflamm. (2015) 2:e109. doi: 10.1212/NXI.0000000000000109 PubMed Abstract | CrossRef Full Text | Google Scholar 23. [frontiersin.org]

After a negative lab and infectious workup, a punch biopsy showed dense eosinophilic infiltrate in the dermis as well as “flame figure” formation. [jofskin.org]

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]

On top of the renal amyloidosis his medical workup revealed presence of sensorineural hearing loss (up to 30 dB in frequency of 6,000 Hz). Within 2 days of anakinra therapy all his systemic features disappeared. [frontiersin.org]

• Neutrophilia

  Initial workup demonstrated an elevated white blood cell count (24.6 WBCs x 109/L) with neutrophilia (18.6 x 109/L) and eosinophilia (3.6× 109/L), as well as elevated inflammatory markers (CRP of 60.86 mg/L and ESR of 33mm/hr). [tandfonline.com]

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]

Disease The choice of treatment is dependent on the extent of disease. [dermatologyadvisor.com]

Dapsone should be used in the treatment of Wells' syndrome in lieu of corticosteroids. [moh-it.pure.elsevier.com]

Other treatments used may include: Topical corticosteroid Ciclosporin Dapsone Methotrexate. Treatment of an associated trigger, such as cessation of an implicated drug, can lead to complete resolution. [dermnetnz.org]

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]

Outlook / Prognosis What is the prognosis for those with Wells’ syndrome? The outlook for people with the Wells’ syndrome is very promising. Flare-ups usually clear within a matter of weeks to months, usually without any lasting scars. [my.clevelandclinic.org]

Nevertheless, the IL-6 inhibitor, tocilizumab, proved effective, achieving the total remission of nephrotic syndrome associated with AA secondary amyloidosis, changing the bleak prognosis of this disease. [reumatologiaclinica.org]

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]

Abstract Eosinophilic cellulitis is an inflammation of, until now, unknown etiology that was first described by George Wells in 1971. Its dominating histological hallmarks are so-called "flame figures" and an eosinophilic infiltrate. [ncbi.nlm.nih.gov]

Summary Epidemiology Approximately 80 cases have been described in the literature. Clinical description The lesions are usually filled with fluid, tender and progress to form hardened plaques of edema and erythema. [orpha.net]

Andrews’ diseases of the skin: Clinical dermatology. 2006. pp. 144(A review of the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, and treatment of Wells syndrome.) Wolverton, SE. [dermatologyadvisor.com]

[…] 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]

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]

It is mainly observed in adults but also in children.[1][3] Pathophysiology The pathogenesis of Wells syndrome is obscure. [statpearls.com]

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]