No Ways Tired

Thursday, November 15, 2012

Wells' Syndrome.... it's a flare up via 4 yrs!!!

Itching, itching, and mild swelling... annoying deep skin pain!!


Definition

Wells syndrome is a rare condition of unknown cause. It is also called ‘eosinophilic cellulitis’.

What does Wells syndrome look like?

Typically the rash is preceded by itching or burning skin and consists of markedly swollen nodules and plaques (lumps) with prominent borders. The patches are usually bright red at first, frequently looking like cellulitis, then fade over four to eight weeks, leaving green, grey or brown patches. They can blister. The rash most commonly occurs on the limbs, but may also affect the trunk.
The patient often feels very tired and has a fever in approximately 25% of cases.

Recurrent cellulitis followed by an eruption of skin lesions that are usually brawny, filled with fluid, and heavily infiltrated by eosinophils and histiocytes. Also called eosinophilic cellulitis.

A blood count may reveal increased numbers of white blood cells called eosinophils – these are often associated with allergy or insect bites.
The diagnosis of Wells syndrome can be established by a skin biopsy finding of typical histopathological features with many eosinophils and characteristic ‘flame figures’. However, flame figures are not diagnostic of Wells syndrome and can be seen in other conditions that have increased numbers of eosinophils.
An important part of the management of patients with Wells syndrome is to exclude underlying causes such as parasitic disoders (e.g. a worm infestation) or an allergic contact dermatitis with the help of the appropriate tests.

Treatment

Oral corticosteroid treatment with prednisone can lead to a dramatic improvement within days and the course is typically tapered over one month. Other treatments include minocycline, dapsone, griseofulvin, ciclosporin and oral antihistamines.
Mild cases may respond to topical steroid therapy alone. 

Approach Considerations

There are numerous treatment options for Wells Syndrome (eosinophilic cellulitis), including the use of topical corticosteroids, calcineurin inhibitors, griseofulvin, H1 antihistamines, cyclosporine, dapsone, and systemic corticosteroids.[38]
Systemic corticosteroids are the most effective treatment, but they may lead to corticosteroid dependence.
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I'm a Rare Disease Person....in addition to diabetes huh?


Wells Syndrome:  Background

In 1971, George Wells first described this syndrome as a recurrent granulomatous dermatitis with eosinophilia.[1] Wells and Smith renamed it eosinophilic cellulitis in 1979.[2]
Wells syndrome (eosinophilic cellulitis) is an uncommon condition of unknown etiology. The presentation usually involves a mildly pruritic or tender cellulitis-like eruption with typical histologic features characterized by edema, flame figures, and a marked infiltrate of eosinophils in the dermis.[3] Papular and nodular eruptions at the clinical presentation have also been reported.[4, 5] The condition can recur and may be preceded by a pruritic papular eruption. Although Wells syndrome is usually sporadic, some familial cases have been reported.
One study showed the successive occurrence of vasculitis, Wells syndrome, and Sweet syndrome in a patient. This finding suggests that there is an overlap between these diseases.[6] Another report describes a dominant syndrome consisting of eosinophilic cellulitis, mental retardation, and abnormal body habitus in one family.[7]

Pathophysiology

At least some cases of Wells syndrome (eosinophilic cellulitis) may represent hypersensitivity to an arthropod bite or sting. An impressive response of peripheral lymphocytes to mosquito salivary gland extracts has been documented in some patients with Wells syndrome.[8] A dermal infiltrate of histiocytes, eosinophils, and eosinophilic granules occurs between collagen bundles, which forms the classic flame figures. The eosinophilic infiltrate is almost always restricted to the epidermis and the dermis, but it has also been found in the subcutaneous tissue and the underlying muscle. The location of the infiltrate is correlated with the different clinical features.
In one study, immunophenotyping of peripheral T cells revealed an increased proportion of CD3+ and CD4+ T cells.[9] These lymphocytes spontaneously release significant amounts of interleukin 5 (IL-5); this finding suggests that activated T cells may be involved in the pathogenesis of blood and tissue eosinophilia. The eosinophils then degranulate in the dermis, causing edema and inflammation.[10]
With immunofluorescent stains, eosinophil major basic protein is identified in the granules of the flame figures. On electron microscopy, the collagen fibers are intact; this finding suggests that an initial degeneration of collagen is not a factor in initiating the formation of flame figures.

Etiology

The etiology of Wells syndrome (eosinophilic cellulitis) is unknown. Wells syndrome may be due to drugs, various infections, and, possibly, nonhematologic malignancies as trigger events.[11, 12]
Wells syndrome is usually sporadic, but some familial cases have been described. Suggested precipitating factors include the following:
  • Arthropod bites and stings, including those of the honeybee[13]
  • Cutaneous viral infections; there is a possible link between parvoviral infection and Wells syndrome[14]
  • Cutaneous parasitic infestations, including toxocariasis,[15, 16] ascariasis,[17] and onchocerciasis[18]
  • Leukemia
  • Myeloproliferative disorders
  • Atopic dermatitis
  • Fungal infections
  • Hypersensitivity reactions to medications or metals, including metallic alloy implants[19]
  • Churg-Strauss syndrome: This syndrome has been associated in a few patients[20] ; these reports are noteworthy for the presence of bullae and of antineutrophil cytoplasmic antibodies[21] ; only a few other autoimmune diseases have been associated, including a case report of systemic lupus erythematosus[22] ; sometimes, differentiating between these disorders can be challenging[23]

Epidemiology

Wells syndrome (eosinophilic cellulitis) is rare. Only about 80 cases have been reported worldwide.
Wells syndrome usually affects adults, but it has been known to occur in children.[24, 25, 26, 27] In one case series of 19 patients, the classic plaque-type presentation was the most common variant found in children, whereas the annular granuloma–like variant was the most common variant in adults.[28]

Prognosis

The prognosis for patients with Wells syndrome (eosinophilic cellulitis) is excellent. It tends to resolve in weeks or months, usually without scarring. It occasionally recurs. In these recurrent cases, it can take years to ultimately resolve.
 
 Contributor Information and Disclosures
Author  Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School 

Additional Contributors   David F Butler, MD Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.
Rosalie Elenitsas, MD Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting
Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc
Disclosure: Nothing to disclose.
References
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  3. Brehmer-Andersson E, Kaaman T, Skog E, Frithz A. The histopathogenesis of the flame figure in Wells' syndrome based on five cases. Acta Derm Venereol. 1986;66(3):213-9. [Medline].
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  27. Powell JG, Ramsdell A, Rothman IL. Eosinophilic cellulitis (Wells syndrome) in a pediatric patient: a case report and review of the literature. Cutis. Apr 2012;89(4):191-4. [Medline].
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  29. Spinelli M, Frigerio E, Cozzi A, Garutti C, Garavaglia MC, Altomare G. Bullous Wells' syndrome associated with non-Hodgkin's lymphocytic lymphoma. Acta Derm Venereol. 2008;88(5):530-1. [Medline].
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  32. Falagas ME, Vergidis PI. Narrative review: diseases that masquerade as infectious cellulitis. Ann Intern Med. Jan 4 2005;142(1):47-55. [Medline].
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  34. Moossavi M, Mehregan DR. Wells' syndrome: a clinical and histopathologic review of seven cases. Int J Dermatol. Jan 2003;42(1):62-7. [Medline].
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  36. Peters MS, Schroeter AL, Gleich GJ. Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells' syndrome. Br J Dermatol. Aug 1983;109(2):141-8. [Medline].
  37. Aberer W, Konrad K, Wolff K. Wells' syndrome is a distinctive disease entity and not a histologic diagnosis. J Am Acad Dermatol. Jan 1988;18(1 Pt 1):105-14. [Medline].
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  40. Brown J, Schwartz RA. Wells' syndrome (eosinophilic cellulitis). Cesko-Slovenska Dermatol. 2002;77:261-263.
 
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