Wells syndrome

A recurrent granulomatous dermatitis with eosinophilia.

Also known as eosinophilic cellulitis.

Of unknown etilology.

Characterized by erythematous plaques resembling cellulitis.

Typically patients have multiple occurrences.

Has seven clinical variances: plaque type, annular granuloma-like, urticaria-like, papulovesicular, h bullous, papulaonodular, and fixed drug eruption like.

Clinically usually begins with a prodromal burning or pruritus, followed by erythematous plaques with red and violaceous border marked edema.

Lesions remains swollen for days-weeks and generally spontaneously subside after weeks-months.

Peripheral eosinophilia is present in about 50% of patients.

Histologically acute phase is characterized by dermal edema and leukocyte infiltrates with predominately eosinophilia.

Wells syndrome is a recurrent granulomatous dermatitis with eosinophilia.

Renamed as eosinophilic cellulitis.

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.

May present as papular and nodular eruptions at the clinical presentation

The condition can recur and may be preceded by a pruritic papular eruption.

Usually sporadic, some familial cases have been reported.

There is an overlap between Wells syndrome and Sweet syndrome.

Some cases may represent hypersensitivity to an arthropod bite or sting.

A significant response of peripheral lymphocytes to mosquito salivary gland extracts has been documented in some patients with Wells syndrome.

A dermal infiltrate of histiocytes, eosinophils, and eosinophilic granules occurs between collagen bundles.

The eosinophilic infiltrate is almost always restricted to the epidermis and the dermis, but it may occur in subcutaneous tissue and the underlying muscle.

The location of the infiltrate is correlated with the different clinical features.

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.

May be due to drugs, various infections, and, possibly, nonhematologic malignancies as trigger events.

Medications linked include antibiotics, anticholinergic agents, anesthetics, nonsteroidal anti-inflammatory agents, thyroid medications, chemotherapeutic agents, thiomersal-containing vaccinations, antitumor necrosis factor agents, and thiazide diuretics.

Vaccines implicated include hepatitis B vaccine, influenza vaccine, tetanus vaccine, tetanus-diphtheria vaccine, and triple-antigen vaccine.

Usually a sporadic process, but some familial cases have been described.

Precipitating factors include: Arthropod bites and stings, cutaneous viral infections, cutaneous parasitic infestations, including toxocariasis, ascariasis, and onchocerciasis, Leukemia, Myeloproliferative disorders, Atopic dermatitis, Fungal infections, Giardiasis, Hypersensitivity reactions to medications or metals, Churg-Strauss syndrome, and autoimmune diseases.

Both Wells syndrome and Churg-Strauss syndrome may have eosinophils and flame figures, suggesting Wells syndrome could represent the initiation of a pathogenetic process fully developed as Churg-Strauss syndrome.

Usually affects adults, but it has been known to occur in children.

Plaque-type presentation is the most common variant found in children, whereas the annular granuloma–like variant is the most common variant in adults.

Prognosis is excellent, as it tends to resolve in weeks or months, usually without scarring.

Usually, patients report pruritus or a burning sensation, which is followed by erythema and edema.

Occasionally, papular and nodular eruptions may be seen first.

Typically, a tender or mildly pruritic cellulitis-like eruption occurs.

The clinical presentation may also include annular plaques, vesicles and bullae and urticaria.

Systemic symptoms, including asthma, arthralgia, and fever, may occur.

Although long-term sequelae usually do not result.

Owever, reticular pigmentation and scarring alopecia may occur.

Rarely, is associated with life-threatening diseases such leukemia and lymphoma.

The lesions in patients progress over a few days to become large, indurated plaques of edema and erythema, with violaceous edges and no collar.

The lesions may last for several weeks.

The lesions gradually darken from bright red to slate blue.

Complete resolution of the lesions is typical.

Lesions usually resolve without scarring.

The plaques lesions can occur anywhere on the skin surface

Lesions may be solitary or multiple.

The clinical features seem to depend on the location of the infiltrates in the dermis.

Differential Diagnoses include:

Allergic Contact Dermatitis


Chronic Urticaria

Dermatologic Manifestations of Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome)

Drug Eruptions

Drug-Induced Bullous Disorders


Granuloma Annulare

Insect Bites

Lyme Disease

Pediatric Hypereosinophilic Syndrome

Usually diagnosed on the basis of the characteristic histopathologic findings in a skin biopsy specimen.

Peripheral blood and bone marrow eosinophilia are usually present.

In peripheral blood, an eosinophil cation protein (ECP) and IL-5 levels can be increased.

Skin biopsy shows a dermal infiltrate of eosinophils, histiocytes, and eosinophil debris between collagen bundles that forms flame figures.

In the acute early phase, the dense infiltrate of degranulating eosinophils is usually located in the epidermis and the dermis, although it occasionally extends into the subcutaneous tissue and the underlying muscle.

Vesiculation and blisters can occur that contain eosinophils that are predominantly subepidermal and, occasionally, multiloculated and spongiotic.

After weeks, flame figures are seen, along with a palisade of histiocytes and giant cells around some collagen fibers.

The histopathologic findings of eosinophilia, histiocytes, and flame figures are characteristic of Wells syndrome, they are also found in other conditions, including bullous pemphigoid, eczema, tinea infection, dermatitis herpetiformis, scabies, and insect bites.

Treatment options for Wells Syndrome include: topical corticosteroids, calcineurin inhibitors, griseofulvin, H1 antihistamines, cyclosporine, dapsone, and systemic corticosteroids.

Systemic corticosteroids are the most effective treatment.

Medications used for the treatment include: antifungals, antibiotics, such as dapsone, immunosuppressants, such as cyclosporine and cortisone, and H1 receptor antagonists.

Leave a Reply

Your email address will not be published. Required fields are marked *