Hypereosinophilic Syndrome:Two Cases Reports And Literature Review

Background: Hypereosinophilic syndrome(HES)is a group of diseases ofunknown etiology,characterized by blood and bone marrow eosinophils persistentincrease and tissue infiltration of eosinophils. HES is a rare disease in clinical,Apart from increased eosinophils, HES is often accompanied by heart, lungs, skin,gastrointestinal, nervous system and other organ damage.Due to the different organsand degree of involvement, the clinical manifestations are complex and diverse, thefirst visit is often difficult to diagnose.Most patients with the disease admitted tohospital for the outstanding performance of a system damage or increasedeosinophils.The diagnosis requires careful physical examination, especially the skin,liver, spleen, lymph nodes, the cardiovascular system and respiratory system.In orderto control organ damage and prolong survival purposes,We should strive for earlydiagnosis and early treatment.Methods: This test reports two cases of hypereosinophilic syndrome with skinlesions for the outstanding performance treated in our department.Case presentation:Case1. A65-year-old man was admitted to the department of dermatology inJanuary2013for the whole body rash with itching for six months. Six months ago, hedeveloped pruritic red maculopapule on neck and hands without obvious incentive.He was given treatment with antihistamines and calamine lotion, there was nosignificant improvement in the condition. Three months ago, similar lesions appearedon the trunk and limbs with severe itching. He went to many hospitals, giventreatment with antihistamines, glycyrrhizin, BCG polysaccharide nucleic acid,hormonal ointment for external use and so on. The effort was not obvious. The lesionsbecame thick,the color became purple brown and brown. For further treatment, hewas admitted to our department. Since he got sick,there was no fever, no cough, nosputum, no abdominal pain, no diarrhea, no swelling and pain in joint.Physical examination revealed lymphadenectasis in bilateral axilla，bean big, smooth, soft,moveable, without adhesion to surrounding tissue and without tenderness. The wholebody presented densely purple brown and brown maculopapule, about rice to soybeanbig, and scattered scratches. Laboratory examination: Blood test showed WBC8.70×109/L, eosinophils proportion22.91%、count1.99×109/L, hemoglobin130g/L. Chest CT showed multiple lymphadenectasis in bilateral axillas. Bone marrowcells examination revealed proliferation of granulocyte was active, eosinophils21.5%,no abnormal cell morphology on various stages, suggesting hypereosinophilicsyndrome. The Skin biopsy showed infiltration of eosinophils and lymphocytesaround dermal blood vessels. Diagnosis: Hypereosinophilic syndrome.Case2. A80-year-old man was suffered rash with itching on the whole body forsix months. He was admitted to the department of dermatology in January2013because the condition aggravated one week ago. Six months ago, he developed redpapules the size of rice on scalp without obvious incentive. He was given treatmentwith cetirizine, vitamin B1in a hospital, the condition somewhat mitigated. Fivemonths ago, similar lesions generalized to the whole body with severe itching. Hewent to many hospitals, given treatment with loratadine, calcium gluconate, transferfactor, mometasone furoate and so on. The effort was not obvious. He was giventreatment with chlorpheniramine, dexamethasone (dose unknown) by himself, thecondition somewhat mitigated. Then he came to our department for treatment. Bloodtest showed eosinophils proportion18.21%、count0.87×109/L. He was diagnosed as“generalized eczema”, then given treatment with diphenhydramine, chlorpheniramine,levocetirizine, mizolastine, vitaminC, glycyrrhizin, hormonal ointment for externaluse and so on, the condition improved markedly. Soon after,the whole bodydeveloped deflushing and desquamation with severe itching. He came to ourdepartment for treatment again.Blood test showed eosinophils proportion32.11%、count2.44×109/L, and the skin biopsy showed subacute dermatitis. He wasdiagnosed as “erythroderma”. He was given treatment with diphenhydramine,chlorpheniramine, levocetirizine, tripterygium glycosides, glycyrrhizin, thymopentin,Thymopentin, hormonal ointment for external use, NB-UVB and so on,the conditionimproved markedly after treatment. One week ago, flushing aggravated of the wholebody. Erythemas and papules appeared in and behind both ears. For further treatment,he was admitted to our department. Since he got sick, there was no fever, no cough, no sputum, no abdominal pain, no diarrhea, no swelling and pain in joint. Physicalexamination revealed lymphadenectasis in bilateral axilla and groin，bean big, smooth,soft, moveable, without adhesion to surrounding tissue and without tenderness. Thewhole body presented flushing, swelling, with chaff-like scales, and a lot of scratches,a small amount of leakage on some scratches, a number of miliary size erythemas andpapule in and behind both ears and on scalp. Laboratory examination: Blood test showed WBC10.84×109/L, eosinophils proportion35.91%、 count3.75×109/L,hemoglobin104g/L. Chest CT showed multiple lymphadenectasis in bilateral axillasand mediastinum. Localized superficial lymph node ultrasound showedlymphadenectasis in bilateral groin.Bone marrow cells examination revealedproliferation of granulocyte was active, eosinophils21.5%, no abnormal cellmorphology on various stages. The Skin biopsy showed hyperkeratosis andparakeratosis, epidermal hypertrophy, infiltration of eosinophils and lymphocytesaround dermal blood vessels, some cells moved into the skin. Diagnosis:Hypereosinophilic syndrome.Conclusion:1. HES is clinically rare, due to the involvement of different tissues and organs,clinical manifestations are complex and diverse.There is no specific of the symptomsand signs, and many reasons that could cause the reactive or consequential increase ineosinophils. So it is often misdiagnosed on the first diagnosis. Clinicians should takeit seriously.2. We need systematic physical examination and laboratory examination for thediagnosis. It is specifically important defining the reasons for the increase ofeosinophils.3. Early diagnosis and early treatment help to control organ damage and prolongsurvival.