Hypereosinophilic syndrome - Skin in Adult
- Peripheral eosinophilia (>1.5 x 109/L) for more than 6 months
- Absence of other causes (ie, allergy, parasitic infections, connective tissue disease, and/or malignancy)
- Multisystem involvement
- Lymphoproliferative HES is associated with abnormal clones of T-cells producing Th-2 cytokines, specifically interleukin-5, which results in increased eosinophil production. These patients are at increased risk of developing lymphoma.
- Myeloproliferative HES is associated with the FIP1L1-PDGFRA fusion gene (encoding an activated tyrosine kinase). This genetic mutation is found in various forms of myeloproliferative HES including eosinophilic leukemia.
- Other eosinophilic disorders: eosinophilic vasculitis; eosinophilic gastritis; episodic angioedema with eosinophilia (EAE); nodules, eosinophilia, rheumatism, dermatitis, and swelling (NERDS) syndrome; etc.
Morbidity and mortality from HES is variable. This condition may be quiescent or rapidly fatal from heart failure secondary to progressive restrictive cardiomyopathy. Malignant transformation to eosinophilic leukemia also carries a poor prognosis.
For more information, see OMIM.
D72.1 – Eosinophilia
128835008 – Hypereosinophilic syndrome
- Angiolymphoid hyperplasia with eosinophilia – Look for papules, plaques and nodules in the head and neck region.
- Eosinophilic granulomatosis with polyangiitis – Look for allergic rhinitis, asthma, peripheral neuropathy, and vasculitis.
- Eosinophilic leukemia – Look for bone marrow sample with leukemic blast cells and eosinophilic differentiation.
- Eosinophilia-myalgia syndrome – Look for flu-like and neurological symptoms and a history of L-tryptophan supplementation.
- Eosinophilic cellulitis (Wells syndrome) – Look for bright-red to slate-blue papules, plaques, or wheals and "flame figures" on skin biopsy.
- Eosinophilic fasciitis – Look for peau d'orange appearance to the skin and a history of strenuous exercise.
- Eosinophilic pustular folliculitis (Ofuji disease) – Look for pruritic folliculitis in HIV patients.
- Eosinophilic ulcer – Look for mucosal (usually oral) ulcers in areas of chronic trauma.
- Kimura disease – Look for painless unilateral cervical lymphadenopathy in Asian males.
- Parasitic infections (eg, strongyloidiasis)
- Drug reactions