A 9 years old with skin rash and eosinophilia

Dr Ira Shah

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A 9 year old with skin rash and eosinophilia

Dr Ira Shah.

Medical Services Department, Pediatric Oncall, Mumbai.

ADDRESS FOR CORRESPONDENCE
Dr Ira Shah, Medical Sciences Department, Pediatric Oncall, 1, B Saguna, 271, B St. Francis Road, Vile Parle {W}, Mumbai 400056.

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Clinical Problem
Case Report:- A 9 year old boy presented with multiple papular eruptions on the face, abdomen and limbs since 6 months. He had no itching, fever or bleeding or failure to thrive. He had been treated for a thigh abscess at 8 years of age for which drainage was required. On examination, apart from the papular eruptions, all other findings were formal. He had one brother and 3 sisters and all were well. Investigations showed:

• Hemoglobin = 12.4 gm/dl
• WBC count = 12,400/cumm (60% polymorphs, 20% lymphocytes, 20% eosinophils)
• Platelet count = 1,70,000/cumm
• Chest X-Ray = Right lower zone pneumatocele
• Ultrasound abdomen = Normal
• Urine & stool examination = Normal
• Skin biopsy = CD3 and CD43 deposits in epidermis without CD20 deposits suggestive of lymphocytic infiltration.

What is the diagnosis? What further investigations should be done?

Discussion
Expert’s opinion:- Dr. Ira Shah

This child seems to have presented with chronic papular eruptions over the skin with eosinophilia. Hence allergic conditions should be ruled out. However, allergic conditions usually lead to itching which in this child was not present. In that case, one must consider a possibility of Hyper IgE syndrome (JOB’S SYNDROME). Hyper IgE disorder is characterized by recurrent staphylococcal abscesses, papular eruptions over skin. It is characterized by eosinophilia and increased level of serum IgE (> 2,000 mg/dl). It is a congenital immunodeficiency with X-linked dominant inheritance pattern and hence seen in boys. This male child did have an abscess at 8 years of age and also had pneumatocele in the Chest X-Ray suggestive of a probable staphylococcal infection. Investigations in form of serum immunoglobulins including serum IgE levels are required. The child’s serum IgE was 3,700 mg/dl and serum IgG, IgM & IgA were normal. A repeat serum IgE after 2 years was 7,600 mg/dl. Thus the diagnosis in this child is Job’s syndrome.

Compliance with ethical standards

Funding: None

Conflict of Interest: None

Cite this article as:
Shah I. A 9 years old with skin rash and eosinophilia. Pediatr Oncall J. 2008;5: 105.