|Year : 2000 | Volume
| Issue : 6 | Page : 306-307
The Hypereosinophilic Syndrome
Dipti Desai, Ram Malkani, Vijay Aswani
Source of Support: None, Conflict of Interest: None
A 64-year old woman patient with dry pruritic rash, oral ulcers, persistent cough and significant weight loss was investigated and proved to be a case of hypereosinophilic syndrome.
Keywords: Hypereosinophilic syndrome, ′HES′
|How to cite this article:|
Desai D, Malkani R, Aswani V. The Hypereosinophilic Syndrome. Indian J Dermatol Venereol Leprol 2000;66:306-7
| Introduction|| |
Eosinophil is a motile phagocytic cell, engaged in exocytosis, a process whereby the contents of cytoplasmic granules are released extracellularly. Stimulated eosinophils are capable of an oxidative burst and can release a number of oxidative products viz. hydrogen peroxide, superoxide ions and hydroxide radicals. Such an eosinophil undergoes morphological alterations. Increased expressions of membrane receptor for IgG, IgE and c3 b have been found on such eosinophils. An eosinophilic response may be a common finding associated with several pathological conditions viz. allergic states, parasitic infestations, bacterial and mycobacterial infections, malignancies and autoimmune diseases. A multisystem involvement and significant morbidity and mortality have been described. The involvement of heart, lungs, nervous system and skin have been given a variety of names, including eosinophilic leukemia, disseminated eosinophilic collagen disease, Loeffler's fibroblastic endocarditis with eosinophilia and pulmonary infiltration with eosinophilia. These entities were grouped together under the heading of hypereosinophilic syndrome (HES) and viewed as a spectrum of diseases with eosinophilic leukemia at one end and relatively symptomatic patients with skin lesions and heart disease at the other.,
HES has been reported in individuals aged 580 years with highest incidence between 41-50 years. The three major criteria for diagnosing HES are:
1) Persistent eosinophilia of 1500 eosinophils/ mm for more than 6 months. 2) No specific known cause for eosinophilia and 3) Signs and symptoms of organ involvement.
The presenting signs and symptoms include anorexia, weight loss, dyspnea, fever, cough, chest pain, pruritic rash, neurologic abnormalities and arthralgias. Mucosal erosion can be a presenting feature of HES like in the present case.
50% of the patients suffer from skin manifestations. Skin lesions usually pruritic may be either erythematous macules, papules and nodules or urticaria, angioedema and dermographism. Additional lesions may include hyperpigmented macules, ulcerated nodules and vesicles, scaling, serpiginous lesions, subcutaneous nodules and palpable purpura. Lesions are more often located on the extremities followed by trunk and the face. Patients with a more benign course of HES usually have angioedema, urticaria or erythematous papules.Multiple organ involvement may include cardiovascular, neurologic, pulmonary and gastrointestinal besides skin involvement.
| Case Report|| |
The present case was a 64-year-old unmarried woman who came with complaints of itchy dry rash all over body since 6 months. She complained of mild to moderate fever with an evening rise, dry cough and significant weight loss (10 kgs) since 5-6 months. She also gave history of ulcer on left side of tongue and swelling on left side of neck since 6 months. She was also under treatment for hypertension with enalpril which was changed to amlodipin in view of the skin rash. Incidentally the patient's sister and mother died of breast cancer and father had carcinoma prostate. Cutaneous examination revealed multiple erythematous papular, nodular and excoriated lesions on the face, especially periorbital, neck, trunk, chest, abdomen, both extremities and buttocks. There were two ulcers about 1/2 cm. size, irregularly shaped on left side of tongue. The left cervical gland was tender and palpable.
Her blood Hb was 12 g %; total and differential count. 12,700; N25 L10 E65 and reticulocyte count 9%. Mantoux test was negative. Blood sugar, liver and kidney parameters were within normal limits. Elisa for HIV I, II was negative. ECG was within normal limits. USG showed enlarged fatty liver. Histopathological study of skin biopsy revealed thin epidermis. Dermis showed infiltration around blood vessels with fair numbers of eosinophils and lymphocytes.
In view of her clinical findings and investigations; persistent eosinophilia of 1500 cells/mm3 for more than 6 months and biopsy findings, she was diagnosed as a case of HES. She was started on tabl. Prednisolone 20 mg. tid, along with tabcetirizine and tab-roxithromycin. The patient felt much better about the skin lesions and the respiratory symptoms. However after about 1 month, patient was admitted elsewhere with fever, cough, edema feet and gallop rhythm. ECG at that time showed non-specific T wave changes. Patient succumbed to death due to cardiomyopathy.
| Discussion|| |
Our present case was diagnosed as HES due to a high grade of suspicion. She was otherwise been treated as a case of drug reaction and a case of respiratory disorder. The patient presented with an oral ulcer which has been reported as a presenting feature of HES recently. The sudden downgrading of patient inspite of initial improvement was unexpected.
Multiple organ involvement is the rule in HES. Cardiac involvement is frequent and may be manifested clinically by tachycardia, apical murmurs and congestive failure. Extensive mural thrombosis and involvement of vascular endocardium is a frequent at autopsy. Pulmonary infiltrations occur leading to fribrosis and a defect in lung diffusing capacity. Hepatosplenomegaly is common. There is also a high incidence of CNS involvement characterized by focal neurologic signs, convulsions, delirium and coma. Rarely renal involvement is seen. At autopsy eosinophilic infiltration is found in all organs., In firbroplastic endocarditis with eosinophilia (Loeffler's endocarditis) extensive myocardial fibrosis and mural thrombosis are dominant clinically and pathologically. The various syndromes as well as our case form a spectrum of disorders in which the common denominator is infiltrative eosinophilia., Besides being a case of not-so-often diagnosed HES, the present case is thought provoking to the treating physician about the uncertainty of the disease in prognosis in spite of proper treatment.
| References|| |
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