Hypereosinophilic dermatitis D72.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 28.10.2022

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Synonym(s)

Dermatitis eosinophil; Eosinophilic dermatitis; hypereosinophilic dermatitis; Hypereosinophilic dermatitis

History
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Nier and Westfried, 1981

Definition
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Idiopathic (polyetiological), possibly monoorganic, dermatological "variant" of hypereosinophilia syndrome (minus variant), with a relapsing course of disease characterized by eminently chronic pruritus.

Localization
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Trunk and extremities with recess of head, palmae, plantae, glans penis and vulva.

Clinical features
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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.

Laboratory
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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!

Histology
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Marked superficial interstitial lymphocytic dermatitis with marked eosinophilia. Focal epitheliotropy with little or no spongiosis. No evidence of vasculitis.

Differential diagnosis
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Eosinophilic cellulitis, urticaria, atopic dermatitis, pruritus with ACE inhibitors; neurogenic pruritus.

External therapy
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If no cause can be determined, first apply external weak or, in the short term, also medium-strength glucocorticoid extracts, e.g. hydrocortisone cream R121 or betamethasone cream R029. In the meantime, apply blanched moisturizing topicals (e.g. base cream (DAC), Linola milk) or Lotio alba.

Radiation therapy
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PUVA bath therapy was used with good success.

Internal therapy
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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)
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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.

Literature
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  1. 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
  2. de Graauw E et al.(2015) Eosinophilia in Dermatologic Disorders. Immunol Allergy Clin North Am 35:545-560.
  3. Kaufmann S et al (1987) Recurrent angioedema with eosinophilic dermatitis-minus variant of hypereosinophilia syndrome. Dermatologist 38: 206-209
  4. Mahajan VK et al (2014) Idiopathic hypereosinophilic syndrome: a rare cause of erythroderma. J Dermatol Case Rep 8: 108-114.
  5. Nir MA, Westfried M (1981) Hypereosinophilic dermatitis. A distinct manifestation of the hypereosinophilicsyndrome
    with response to dapsone.Dermatologica 162:444-450.
  6. 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.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 28.10.2022