HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Idiopathic (polyetiological), possibly monoorganic, dermatological "variant" of hypereosinophilia syndrome (minus variant), with a relapsing course of disease characterized by eminently chronic pruritus.
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LocalizationThis section has been translated automatically.
Trunk and extremities with recess of head, palmae, plantae, glans penis and vulva.
Clinical featuresThis section has been translated automatically.
Often rather discrete, variable exanthema with disseminated, barely pinhead- to 0.5cm large, red, usually extremely pruritic, spots, papules and papulovesicles. Not infrequently, a clear lichenification of the lesions is detectable. The leading symptom of the disease is often excruciating itching. Accordingly, scratch erosions are frequently detectable. Eosinophilic organ involvement can be excluded. The transition to a hypereosinophilia syndrome is smooth.
LaboratoryThis section has been translated automatically.
Leading symptom: Persistent or undulating blood eosinophilia >10%. When taking the blood count, it is important to make sure that the patient had not received any external or internal glucocorticoids at the time of blood collection. Multiple controls under strict steroid abstinence are recommended!
HistologyThis section has been translated automatically.
Marked superficial interstitial lymphocytic dermatitis with marked eosinophilia. Focal epitheliotropy with little or no spongiosis. No evidence of vasculitis.
Differential diagnosisThis section has been translated automatically.
Eosinophilic cellulitis, urticaria, atopic dermatitis, pruritus with ACE inhibitors; neurogenic pruritus.
External therapyThis section has been translated automatically.
Radiation therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
If local therapy is insufficiently successful, systemic treatment with glucocorticoids initially 20-40 mg/day p.o. prednisone equivalent is recommended. Continuous therapy with 5-10 mg/day p.o.
Additively methotrexate can be helpful (10-15mg/week) -s. Casuistry.
Alternative: Good therapeutic results of a multicenter study with a "targeted" therapy with mepolizumab, an anti-IL-5 antibody are available. Mepolizumab could provide new therapeutic options for eosinophilic granulocyte-triggered dermatitis in the future (off-label use).
Case report(s)This section has been translated automatically.
A 22-year-old woman has been suffering from a generalized, highly pruritic exanthema for 3 years, which has led to marked lichenifications. The skin lesions presented as extensive, even figured, anular plaques on the trunk and extremities. Abundant scratch excoriations were found.
Laboratory: Leukocytosis with eosinophilia (25%) in peripheral blood, elevated serum IgE levels.
Skin biopsy revealed only a moderately dense perivascular and interstitial dermal infiltrate with eosinophils and lymphocytes. Bone marrow examination revealed myeloid hypercellularity with increased numbers of eosinophils but no atypical cells. Cytogenetic studies revealed no chromosomal abnormalities.
Imaging: no evidence of systemic involvement.
Diagnosis: idiopathic, (monoorganic) hypereosinophilic syndrome (hypereosinophilic dermatitis).
Therapy: oral prednisolone and weekly methotrexate. Under this therapy significant improvement of the symptoms.
LiteratureThis section has been translated automatically.
- Amini-Vaughan ZJ et al (2012) Therapeutic strategies for harnessing human eosinophils in allergic inflammation, hypereosinophilic disorders, and cancer. Curr Allergy Asthma Rep 12: 402-41
- de Graauw E et al.(2015) Eosinophilia in Dermatologic Disorders. Immunol Allergy Clin North Am 35:545-560.
- Kaufmann S et al (1987) Recurrent angioedema with eosinophilic dermatitis-minus variant of hypereosinophilia syndrome. Dermatologist 38: 206-209
- Mahajan VK et al (2014) Idiopathic hypereosinophilic syndrome: a rare cause of erythroderma. J Dermatol Case Rep 8: 108-114.
- Nir MA, Westfried M (1981) Hypereosinophilic dermatitis. A distinct manifestation of the hypereosinophilicsyndrome
with response to dapsone.Dermatologica 162:444-450.
Yadav D et al (2019) Hypereosinophilic dermatitis: generalised lichenification and gyrate erythema as the sole manifestation of idiopathic hypereosinophilic syndrome. BMJ Case Rep 12: e232142.
Incoming links (11)Anti-interleukin-5 therapy; Betamethasone valerate cream hydrophilic 0.025/0.05 or 0.1% (nrf 11.37.); Chronic lymphocytic leukemia; Eosinophilia and skin; Hydrocortisone cream hydrophilic 0.25/0.5 or 1% (nrf 11.36.); Hypereosinophilia syndrome; Hyper-ige dermatitis; Interleukin-5; Mepolizumab; Myeloid neoplasms with eosinophilia ; ... Show all
Outgoing links (9)Betamethasone valerate cream hydrophilic 0.025/0.05 or 0.1% (nrf 11.37.); Eosinophilic cellulitis ; Glucocorticosteroids systemic; Hydrocortisone; Hydrocortisone cream hydrophilic 0.25/0.5 or 1% (nrf 11.36.); Hypereosinophilia syndrome; Mepolizumab; Papel; Puva bath therapy;
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