Hypereosinophilic syndrome

Introduction

The Hypereosinophilic syndrome is defined as an idiopathic continuum of disease having as a common denominator, "Hypereosinophilia", of blood and bone marrow associated with infiltrative eosinophilia of various organs. Rapidly fatal form occurs at one end of the spectrum and benign forms consisting of skin involvement and asymptomatic heart diseases at the other end. This syndrome of unknown etiology occurs 90% in men and 10% in females.[1],[2],[3] We report one case of hypereosinophilic syndrome (HES) in a female.

Case Report

A 40 - year - old female presented with the history of itching of all over body associated with rashes for last 7 days. She also had low grade fever, dry cough, generalized body ache, weight loss, joint pains and swelling over hands and feet for the last two years. She was admitted this time in our hospital for history of loose motions and high grade fever associated with oral ulcers. For her past history, she was diagnosed as a case of allergic dermatitis and was on steroids, anthistaminics and antibiotics. There was no history of worm infestations. There was no history of allergy i.e. any history of hay - fever, eczema, bronchial asthama etc. Family history was also insignificant. She had history of reccurence of symptoms whenever she stopped steroid therapy.

One examination the patient was toxic. She was pale. Her blood pressure was 100′/ 70 mm Hg. There was swelling over hands and feet. Skin was dry, scaly, thinkened with papules and nodules over trunk and extremities. Urticarial rash was present all over the body sparing the face and neck.

Liver was 4 cm below costal margin, soft, tender with smooth surface and rounded margin. Spleen was 3 cm below costal margin and non - tender. No other abdominal organomegaly or free fluid was found. Chest was clear. In nervous system, there were signs of peripheral neuropathy although no focal neurological deficit was noted.

Hemoglobin was 12 gm/dl, White cell count was 66x109/L, with 69% eosinophils, 1 1 % polymorphs, 17% lyphocytes and 3% monocytes and 3% monocytes. There were no blasts in the peripheral blood. ESR was 53 mm in first hour, the platelet count was maintained at 200x 109/L, and absolute eosinphil count was 46x 109/L. After one month of therapy with steroids, white cell count was 51 x 109/ L with 62% eosinophils, 15% polymorphs and 23% lymphocytes. The platelet count was same. Absolute eosinophil count was 31x 109/L. Bone marrow aspiration showed, marked erythroid hyperplasia, moderate myeloid hyperplasia, massive eosinophilia and adequate megakaryocytes. After 3 months of therapy, Hb was 13 gm/dl, white cell count was 11 x 109/L with 48% polymorphs, 28% lymphocytes, 22% eosinophils and 2% monocytes. Absolute eosinophil count was markedly reduced and maintained at 2x109/L. After stopping the corticosteroid therapyfor 10 days, the WBC count was 76x109/L with 67% eosinophils, 17% lymphocytes, 12% polymorphs and 4% monocytes. Now absolute count of eosinophils, 17% lymphocytes, 12% polymorphs and 4% monocytes. Now absolute count of eosinophils became higher and was maintained at 50x 109/L. Serum potassium was 3.4 Meq/L, serum IgE was 500 IU/ml (normal value in adults is 14 IU/ml). Rheumatoid factor was negative. Skin scraping for fungus and Hansen′s bacillus was negative. Chest X-ray was normal. Ultrasonography of the abdomen revealed hepatospi enomegaly with normal ecostructure. Electrocardiogram revealed sinus tachycardia. Echocardiography was normal. Skin biopsy taken from the lesion in the hand showed eosinophilic infiltration with few lymphocytes, perivascular infiltration in dermis region. ACTH suppression test was normal.

Discussion

This report is the case study of a female patient who presented with the skin lesions of exfoliative dermatitis associated with angiodema of face and extremities. These lesions were present in this patient for last 7 years. The skin lesions in this case were erythematous scaly pruritic patches distributed all over the trunk and extremities which are not pathognomic of HES. These are present in only 57% of the cases, as reported by National Institute of Health, Clinical Centre.[1] According to them, skin lesions may be the only clinical evidence of disease in an otherwise asymptomatic patient.

It is generally observed that HES is composed of several closely related disease entities. The syndrome is characterized by persistent and prolonged eosinophilia with primary hematological, cardiac, neurological and dermatological abnormalities. The etiology of HES is not known till today. It is postulated that in majority of cases, the hypersensitivity reaction to some unknown type of antigen occurs. Some of these patients are likely to develop "eosinophilic leukemia" which is a fatal disease. However, the diagnosis of HES is cimpatible with long survival as in our patient who survived for 7 years.

A recent review,[4] suggested that 27% of these reported cases of HES had skin lesions. Skin lesions have been described by Evans and Nesbit,[5] and Zetterstrom.[6] In this patient, the skin lesions and eosinophilia improved for a short time with oral corticosteroid therapy and these lesions recurred after the therapy was stopped and relapse occured within 4 months.

Angiolymphoid hyperplasia with eosinophilea (Kimura′s disease), is another condition which chiefly occurs in male subjects and is another disease associated with blood eosinophilea and skin lesions. HES differs from this disorder in histological and clinical manifestations and has no ralationship with each other. The prognosis in Kimura′s disease is excellent and treatment often includes excision, radiation, interlesional steroids and carbon-dioxide laser.

In this patient, apart from exfoliative dermatitis as a skin marker associated with eosinophilia, hepatosplenomegaly and peripheral neuropathy, no other involvement was present. The cardiac profile including echocardiagraphy was normal in this patient.

Although the etiology of HES is unknown, recent experimental studies have provided some observations that may partially explain the migration and infiltration of eosinophils in various organ sites. Products of complement activation (C3 and C5a) appear to attract eosinophils and polymorph cells[7] which may account for mixed cell population seen on biopsy specimen. Antigen antibody response in the mast cells and basphils may produce marked eosinophilia and chemical mediators released from these cells, for example, eosinophil chemotactic factor and histamine may be resposible for eosinophilia in this disease.′ Recent study suggested that eosinophils may be involved in the modulation of histamine release or repairative process in the damaged connective tissue.[8] The rarity if this syndrome in female patients with the presentation as exfoliative dermattitis, organomegly and peripheral neuropathy warrent us to report this case.