Cellulite eosinophils L98.3

Rare, inflammatory systemic disease (?) of unclear etiology, whose (clinically uncharacteristic) skin infestation with recurrent, itching or burning, deep red solid plaques is the leading clinical symptom. Initially the clinical picture may remind of erysipelas (fever in 1/4 of the cases).

Recurrent course is possible. Later phases are more reminiscent of morphea or mycosis fungoides.

The diagnosis is usually made on the basis of its histological peculiarity (histoeosinophilia with flame figures). Eosinophilic infiltrates of internal organs (eosinophilic pneumonia, pleuritis, pericarditis) are possible but rather rare.

Unexplained; there is increasing evidence that it is not an entity but a histopathological pattern of response to various diseases. stimuli. Pathogenetically, it is based on a tissue reaction to the release of "eosinophil major basic protein" (MBP) with the involvement of leukotrienes.

In individual cases, insect bites, haematological systemic diseases such as leukaemias, lymphomas or solid tumors (described in breast cancer, renal cell carcinoma) are discussed as triggers.

In children, associations with atopias (atopic eczema) have been described; further after vaccinations.

In adulthood, (eosinophilia-inducing) drugs can also be a trigger (see also casuistry). NSAIDs, antibiotics, TNF-alpha-blockers, thyroid drugs have been described as triggering agents.

Children (often with signs of atopy) 4-10 years

Adults: 40-60 years

Biphasic course of disease. Acute onset.

• Early stage: First unspecific, but mostly pronounced pruritus, possibly combined with burning; within a few days formation of circumscribed, mostly large-area, sharply defined erythema, dolent swellings and doughy to firm large-area plaques. The surface of the plaques may have an orange peel-like texture (see figure). Blistering or bubble formation is rare.
• Fatigue, tiredness and arthralgias can accompany this. The initial stdium can resemble acute erysipelas.
• Late stage: Granulomatous dermatitis with eosinophilia. Plaques that persist for weeks and become increasingly firm, doughy, firm consistency; surface smooth, possibly formation of livid-grey, morphea-like indurations, also strongly itchy prurigo-like papules or nodes. Progression in stages, so that several stages can exist side by side.
• Associated symptoms: Facial paralysis possible. Other systemic involvement is rare.

Focally dense, perivascularly and interstitially stored infiltrates, almost exclusively consisting of eosinophilic and (few) neutrophilic granulocytes, which may also be stored around adnexa. Focal, polygonally limited, eosinophilic flame figures in the dermis.

Notice! "Flame figures are formed by coating collagenous fibres with eosinophilic granules. Circumscript collagen necrosis, eosinophilocytoclasia, granulomatous reaction.

Clinical:

• Erysipelas
• circumscribed scleroderma
• dermatitis herpetiformis
• Urticaria vasculitis
• Urticaria

Histological:

• Churg-Strauss syndrome (sign of vasculitis)
• Arthropod reaction (wedge-shaped eosinophilic infiltrate)

• Glucocorticoids such as prednisolone (e.g. Decortin H) initial 100 mg/day, slow dose reduction depending on skin findings, passagere maintenance dose with 5 mg/day p.o. (dosage below the Cushing's threshold). Additional stomach protection with e.g. Riopan Gel 3 times/day 1 btl.
• In the case of recurrent disease, the combination of systemic glucocorticodes with DADPS (e.g. Dapson Fatol) has proven to be effective, starting with 100 mg/day DADPS p.o. Rapid dose reduction within the next few weeks to a maintenance dose of 50 mg/day p.o. according to the skin findings.

Untreated, spontaneous remission occurs after several weeks to months. Chronic recurrent course over many months is possible. Patients with Wells syndrome may develop signs of eosinophilic systemic vasculitis ( e.g. eosinophilic granulomatosis with polyangiitis).

A 57-year-old female patient reported on intensively itchy, blurred fibrously bordered, high red, moderately doughy-consistent, 10x8cm large, surface smooth plaques on the left thigh, which had been present for 1-2 days and had appeared for the first time. Individual satelite-like papules and plaques were localized in the immediate vicinity of the primary focus. No fever or other AZ disorders. Laboratory findings were leukocytosis (11,000 leukocytes/ml), with distinct eosinophilia (18%) and slightly increased inflammatory parameters. Because of a known but not progressive breast cancer, the patient took tamoxifen.

Histological examination revealed: Focal dense perivascular and interstitial infiltrates almost exclusively of eosinophilic and (few) neutrophilic granulocytes. Detection of eosinophilic flame figures in the dermis.

Therapy: Rapid remission was observed with the use of topical class III glucocorticoids. In the following 2 months 2 relapses occurred in loco, which responded to topical therapy. The blood eosinophilia persisted in unchanged level.

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3. Brun J et al (2015) Groupe de Recherche de la Société française de dermatologie pédiatrique. Wells Syndrome in children and atopy: retrospective study of 11 cases and review of the literature. Ann Dermatol Venereol doi: 10.1016/j.annder.2015.02.017
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