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Year : 1997  |  Volume : 63  |  Issue : 2  |  Page : 117-119

Wells' syndrome

Ajay Chaudhary, Vijay Aswani, Ram Malkani 
 

Correspondence Address:
Ajay Chaudhary


Abstract

Eosinophilic cellulitis/Wells«SQ» syndrome is a rare dermatosis with erythematous, urticarial plaques that become more indurated and eventually have grey blue discoloration. The histopathology is distinctive, with a diffuse infiltrate composed predominantly of eosinophils but admixed with lymphocytes, histicytes and occasionally multinucleated histiocytes. There is dermal edema with so called «DQ»flame figures«DQ» that is composed of collagen focally enveloped with aggregates of eosinophilic granules. These collagen fibres may be surrounded by palisading histiocyes. The course is variable with waxing and waning and eventual spontaneous resolution.



How to cite this article:
Chaudhary A, Aswani V, Malkani R. Wells' syndrome.Indian J Dermatol Venereol Leprol 1997;63:117-119


How to cite this URL:
Chaudhary A, Aswani V, Malkani R. Wells' syndrome. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2019 Nov 15 ];63:117-119
Available from: http://www.ijdvl.com/text.asp?1997/63/2/117/4535


Full Text

 Introduction



Wells' syndrome or eosinophilic celluitis is a rare, recurrent inflammatory dermatosis of uncertain pathogenesis. It was described by Wells in 1971 as "recurrent granulomatous dermatitis with eosinophilia.[1][2] Wells reported 4 patients with distinctive cutaneous lesions, initially resembling bacterial cellulitis and characterised histopatholgicaly by dermal eosinophilia, phagocyte histiocytes and presence of "flame figures".

The term "eosinophilic cellulites" was introduced by Wells and Smith in 1971.[2] The observation of similar histopathologic features in a variety of conditions associated with tissue eosinophilia has led some to question the existence of Wells' syndrome as a distinct entity.[3] Wells' syndrome has been described both in adults and children.[2] Associated disorders and potential precipitating factors include insect bites,[3] onchocerciasis, varicella, mumps, atopic diathesis, fungal infections,[2] drug reactions and myeloproliferative disorders.[1][2]

 Case Report



A previously healthy 14-year-old female was stung by an unknown insect on her left foot. 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. A biopsy specimen from an erythematous papule on the dorsum of the left foot revealed that the epidermis was mildly hyperplastic and and a slight degree of spongiosis was present. Dermis was diffusely infiltrated by eosinophils both perivascular and interstitial. Collagen bundles incrusted with eosinophilic granules characteristic of "flame figures" were seen. Few lymphocytes were also observed around the dermal vessels.

The patient initially was treated with systemic steroids. The patient initially did respond to steroids, but on discontinuing them, the lesions started to increase in size. Later the patient was treated with antihistaminics along with 10 mg of dapsone. The lesions became non-indurated and flat within 10 days of starting treatment. By the 20th day, only postinflammatory hyperpigmentation was seen.

 Discussion



Wells described a clinical and a histopathologic disorder characterised by recurrent granulomatous dermatitis with eosinophilis. Clinical course consists of 2 stages, first or the cellulitic stage being characterised by localised erythema and oedema of the skin, sometimes with blistering and the second or the granulomatous stage charaterised by a gradual resolution of erythema from the centre of the lesions, which remains oedematous and slate colored for several weeks.

Histopathologically, acute stage was characerised by dermal oedema and masses of leucocytes mainly eosinophils. The subacute stage demonstrated dermal eosinophils and histiocytes around angular, focal, fibrinoid masses. The "flame figures" were composed of granules, eosinophilic material adherent to collagen and surrounded by granulomatous inflammation. The late stage reveals resolution of lesions characerised by histiocytic necrobiosis and persistence of "flame figures".

Some authors do not believe that Wells' syndrome is a distinct clinical entity, but rather consider it a histopathologic reaction pattern common to multiple disorders characterised by tissue eosinophilia.[3] Others emphasize that the diagnosis should be reserved for cases with typical features, characterictic histopathology and recurrent course. 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. An abnormal or dysregulated eosinophil response appears to be implicated.[4] The "flame figures" are composed of eosinophilic major basic protein deposited on the collagen bundles.[5] Wells' syndrome is known to respond spontaneously. Although systemic steroids appear to be the only therapeutic modality of benefit in Wells' syndrome, antihistaminics and dapsone have been found to be useful[Figure 1][Figure 2][Figure 3].

References

1Rook A, Wilkinson DS, Ebling FJG. Textbook of dermatology. 5th edition. Oxford : Blackwell, 1992:1838-39.
2Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol 1979;100:101-9.
3Scharr WF, Tauscheck Al, Dickson KB, Melski JW. Eosinophilic cellulitis (Wells' syndrome): histopathologic and clinical features in arthropod bite reaction. J Am Acad Dermatol 1984;11:1043-9.
4Melski JW. Well's syndrome-Insect bite and eosinophilia. Dermatol Clinic 1990;8:287-93.
5Peter MB, Schroetrer Al, Gleich GJ. Immunoflouresence identification of eosinophil granule major protein in the "flame figures" of Wells' syndrome. Br J Dermatol 1983;109:141-8.

 

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