Eosinophilia and skin Grunderkrankung + D72.1

Skin manifestations characterized by eosinophilic infiltrates of the skin (accompanying insect bites, drug reactions, atopic dermatitits or other neoplastic or immmunological misreactions) and/or by hematoeosinophilia.

Eosinophilia is the elevation of the normal value of eosinophilic granulocytes in blood (normal value: 2-4% or 50-500/µl) or tissues. Hematoeosinophilia is present when the absolute number of eosinophils of 500/µl is exceeded (> 50% of total leukocytes).

The formerly used term "hypereosinophilia" (> 1500/µl) is no longer in use and is only used nomenclatorically in the so-called hypereosinophilia syndrome.

• Primary eosinophilia:
  • clonal eosinophilia
  • idiopathic eosinophilia.
• Secondary eosinophilia:
  • reactive eosinophilia.
• Primary eosinophilias include clonal and idiopathic forms. Clonal eosinophilias are predominantly hematologic neoplasms or myelodysplastic and -proliferative disorders.
• Reactive (or secondary) eosinophilias include all conditions in which a short-term or persistent eosinophilia always occurs in association with a detectable disease (e.g., parasitic disease, malignant tumors) or cause (e.g., drugs) of nonclonal origin. Numerous other dermatologic diseases are also classified here.

The following is a list of diseases that are constantly or inconstantly accompanied by blood eosinophilia:

• Allergic diseases (forms of atopic diseases):
  • Atopic eczema
  • Asthma, allergic (see below bronchial asthma)
  • Rhinoconjunctivitis allergica
  • Netherton 's syndrome (see below Ichthyosis linearis circumflexa) - often combined with atopic diseases
  • Urticaria (inconstant, no leading symptom)
  • Erythema exsudativum multiforme (inconstant).
• Parasitic diseases (especially when the parasites or their secretions penetrate into the tissue):
  • Worm infections (for an overview see anthelmintics below)
  • Epizoonoses ( scabies, pediculosis, pulicosis, trombidiosis, cereal scabies)
  • Insect bites caused by hymenoptera.
• Infectious and parainfectious diseases:
  • Special fungal infections ( aspergillosis, coccidioidomycosis)
  • Diseases caused by protozoa (see zoonoses below)
  • Various viral infections ( HIV infection, measles, ringworm)
  • Scarlet fever
  • Spotted fever
  • Acrodermatitis papulosa eruptiva infantilis (Gianotti-Crosti syndrome).
• Blistering dermatoses:
  • Diseases of the pemphigus group (see below Pemphigus)
  • Diseases of the pemphigoid group (see below Pemphigoid)
  • Dermatitis herpetiformis (inconstant, often only histoeosinophilia)
  • Incontinentia pigmenti (Bloch-Sulzberger) (eosinophilia constant in the blister stage)
  • Stevens-Johnson syndrome (eosinophilia inconstant).
• Diseases of the hematopoietic system:
  • Hypereosinophilia syndrome (high eosinophilia is obligate).
  • Hypereosinophilic dermatitis (high eosinophilia is obligate).
  • Episodic angioedema with eosinophilia (Gleich syndrome; constant and marked eosinophilia)
  • Eosinophilic leukemia
  • Erythroderma (with evidence of cutaneous T-cell lymphoma)
  • cutaneous B-cell lymphoma
  • cutaneous T-cell lymphomas
  • Myeloma
  • lymphogranulomatosis, malignant
  • Mastocytosis
  • Histiocytoses, Langerhans cell histiocytoses (e.g. eosinophilic granuloma).
• Collagenoses:
  • Eosinophilic fasciitis (constant)
  • Eosinophilic myalgia syndrome (toxic-oil syndrome)
  • Dermatomyositis (inconstant)
  • Scleroderma, systemic (rather rare).
• Vasculitides:
  • Arteritis temporalis (inconstant)
  • Churg-Strauss syndrome
  • Polyarteritis nodosa, systemic (inconstant)
  • Vasculitis, leukocytoclastic (non-IgA-associated) (mostly histoeosinophilia)
  • Wegener's granulomatosis (eosinophilia may be excessive).
• Endocrine disorders:
  • Addison's disease, M.
• Immunodeficiencies:
  • Episodic angioedema with eosinophilia
  • Selective IgA defect
  • Wiskott-Aldrich syndrome
  • Sarcoidosis (in about 25% of patients)
  • Hyper-IgE syndrome (regular and pronounced).
• Various other diseases:
  • As a paraneoplastic syndrome (e.g., in various solid tumors).
    • Carcinomas of the colon, stomach, ovary, pancreas, cervix uteri, and thyroid; bronchial carcinomas.
  • DRESS
  • Dermatitis, exudative discoid lichenoid (oid-oid disease).
  • Panniculitis, pancreatic
  • Behçet, M.
  • Pustulosis, sterile eosinophilic
  • Post scale azar dermatosis
  • Omenn syndrome
  • Hypermelanosis, nevoid, striate and whorl-shaped
  • Cellulitis, eosinophilic (Wells syndrome)
  • Kimura's disease (angiolymphoid hyperplasia with eosinophilia)
  • Anticonvulsant hypersensitivity syndrome
  • Episodic angioedema with eosinophilia (Gleich syndrome; constant and marked eosinophilia)
  • Erythema neonatorum.

• Medical history (allergies, atopy, family history, medication, vaccinations, travel history)
• Physical examination (which organs are involved, skin, heart, lungs, GIT)
• Laboratory: blood count, ECP, stool tests (parasites), prick, RAST

Notice! A eosinophile count > 1500/ul is rather unusual for allergic diseases. The constellation of haematoeosinophilia and non-specific skin symptoms requires haematological clarification.

A rearrangement of the genes of the "platelet-derived growth-factor receptor" (PDGFR) alpha and beta can be detected in various clonal hemato-eosinophilia. By inhibition of certain cytoplasmic tyrosinases ( imatinib) influenced by this receptor, primary eosinophilia can be successfully treated.

1. Wozel G (2007) Eosinophilia dermatoses. dermatologist 58: 347-360