Eosinophilic cellulitis L98.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 13.10.2022

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Synonym(s)

Dermatitis granulomatous recurrent with eosinophilia; Dermatitis granulomatous with eosinophilia; Eosinophilic cellulite; eosinophilic cellulitis; Eosinophil infiltrate of the skin; Recurrent granulomatous dermatitis with eosinophilia; Wells Syndrome; Wells`syndromes

History
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Miescher, 1945; Wells, 1971

Definition
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Rare, inflammatory systemic disease (?) of unclear etiology, whose (clinically uncharacteristic) skin involvement with recurrent, itchy or burning, saturated red solid (eosinophilic) plaques, is the leading clinical symptom. Initially, the clinical picture may resemble erysipelas (fever in 1/4 of cases).

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

Diagnosis is usually based on its histologic feature (histoeosinophilia with flame figures). Eosinophilic infiltrates of internal organs (eosinophilic pneumonia, pleurisy, pericarditis) are possible but rather rare.

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

In individual cases, insect bites, hematological systemic diseases such as leukemias, lymphomas or solid tumors (described in mammary carcinoma, renal cell carcinoma) are discussed as triggers.

In children, associations with atopy(atopic dermatitis) and vaccination have been described.

In adults, (eosinophilia-inducing) drugs can also be triggering (see also case report). NSAIDs, antibiotics, TNF-alpha blockers, thyroid therapeutics have been described as triggering.

Furthermore, the syndrome was reported after application of a BNT162b2 mRNA Covid-19 vaccine ( Montjoye L et al. 2022).

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

Adults: 40-60 years

Clinical features
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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.

Laboratory
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Eosinophilia (blood, bone marrow), leucocytosis, thrombocytosis possible.

Histology
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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.

Histopathological algorithm of Wells syndrome (lowest common denominator: italics, leading symptoms: bold) varies according to Ratzinger et al. 2105
Accentuated around post-capillary venules
Capillaries recessed
perivascular leukocytoclasia
Damage to endothelial cells
Fibrin in/around vessel walls
Perivascular extravasation of erythrocytes
Edema in the papillary dermis
Collagen degeneration
Eosinophil infiltrates with flame figures, palisade granulomas
No plasma cells or fibrosclerosis

Differential diagnosis
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Clinical:

Histologic:

  • Churg-Strauss syndrome (signs of vasculitis).
  • Arthropod reaction (wedge-shaped eosinophilic infiltrate)
  • Erythema anulare centrifugum (eosinophilia is not predominant)

External therapy
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Blande therapy with lotio alba. If necessary, lotions containing glucocorticoids (e.g. hydrogals, Betnesol V, R030 ).

Radiation therapy
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Possibly try out PUVA therapy.

Internal therapy
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Glucocorticoids such as prednisolone (e.g. Decortin H) initially 100 mg/day, slow dose reduction depending on skin findings, passive maintenance dose with 5 mg/day p.o. (dosage below Cushing's threshold). Additional gastric protection with e.g. Riopan Gel 3 times/day 1 tbl.

In case of recurrent course, the combination of systemic glucocorticodes with DADPS (e.g. dapsone fatol) has proven effective, start with 100 mg/day DADPS p.o. Rapid dose reduction within the next weeks according to the skin findings to a maintenance dose of 50 mg/day p.o.

Progression/forecast
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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).

Case report(s)
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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.

Literature
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  1. Afsahi V et al (2003) Wells syndrome. Cutis 72: 209-212
  2. Brehmer-Andersson E, Kaaman T, Skog E, Frithz A (1986) The histopathogenesis of the flame figure in Wells syndrome based of five cases. Acta Derm Venerol (Stockh) 66: 213-219.
  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
  4. de Montjoye L et al (2022) Eosinophilic cellulitis after BNT162b2 mRNA Covid-19 vaccine. J Eur Acad Dermatol Venereol 36: e26-e28.
  5. Fujii K et al (2003) Eosinophilic cellulitis as a cutaneous manifestation of idiopathic hypereosinophilic syndrome. J Am Acad Dermatol 49: 1174-1177.
  6. Heelan K et al (2013) Wells syndrome (eosinophilic cellulitis): Proposed diagnostic criteria and a literature review of the drug-induced variant. J Dermatol Case Rep 7:113-120
  7. Long H et al (2015) Eosinophilic skin diseases: A Comprehensive Review. Clin Rev Allergy Immunol PubMed PMID: 25876839.
  8. Rajpara A et al (2014) Recurrent paraneoplastic wells syndrome in a patient with metastatic renal cell cancer. Dermatol Online J 20. pii: 13030/qt35w8r1g3.
  9. Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
  10. Sinno H et al (2012) Diagnosis and management of eosinophilic cellulitis (Wells' syndrome): A case series and literature review. Can J Plast Surg 20:91-97
  11. Wells GC (1971) Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc 57: 46-56.
  12. Wells GC, Smith NP (1979) Eosinophilic cellulitis. Br J Dermatol 100: 101-109

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Last updated on: 13.10.2022