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Mr. and Mrs. Ronaldo, first cousins from a Spanish family, welcomed their 4th child Alonzo
and they were thankful that the pregnancy and birth had gone well. Two previous babies, a boy
and a girl, had died within 4 weeks after birth. The other remaining child, a girl, was 10 years
and healthy. However, within a week after birth Alonzo began to have more frequent bowel
movements and developed a scaly rash on his limbs that then spread to his arms, trunk, and face.
Subsequently the rash was accompanied by edema in the tissues producing a wrinkled
appearance of the skin. The two deceased babies had developed a very similar rash. Alonzo also
began to have a dry cough starting in his 3rd week. His parents brought him to the emergency
room at the Mattel Children's Hospital at UCLA. Alonzo's weight, length, and head
circumference were normal. Small blisters were found on his palms and soles and he had yellow
discharge from his eyes consistent with bacterial conjunctivitis. No signs of a middle ear
infection were detected, and the physical examination of his heart and lungs was normal. The
liver and spleen were not enlarged. Alonzo was admitted to the hospital. Blood tests showed that
his hemoglobin was 8.4g/dL (low), his platelet count was 460,000 (slightly elevated), and his
white blood cell count was 8000/l (normal), of which 56% were eosinophils (normal <5%),
23% monocytes (normal 10%), 15% neutrophils, and 6% lymphocytes (normal 50%).
Alonzo's serum IgG level was 55mg/dL (normal 400mg / dL ), IgA and IgM were undetectable,
and IgE was 7200IU/ml (normal <50IU/mL ). A skin biopsy showed that the dermis was
infiltrated with large numbers of eosinophils, lymphocytes, and macrophages. An Xray of
Alonzo's chest showed clear lungs and a normal cardiac shadow; there was no thymic shadow.
After admission, Alonzo developed severe infections with pus from which Staphylococcus
aureus and Candida albicans were cultured and he also developed a thrush with Candida
albicans.

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  • 1. Mr. and Mrs. Ronaldo, first cousins from a Spanish family, welcomed their 4th child Alonzo and they were thankful that the pregnancy and birth had gone well. Two previous babies, a boy and a girl, had died within 4 weeks after birth. The other remaining child, a girl, was 10 years and healthy. However, within a week after birth Alonzo began to have more frequent bowel movements and developed a scaly rash on his limbs that then spread to his arms, trunk, and face. Subsequently the rash was accompanied by edema in the tissues producing a wrinkled appearance of the skin. The two deceased babies had developed a very similar rash. Alonzo also began to have a dry cough starting in his 3rd week. His parents brought him to the emergency room at the Mattel Children's Hospital at UCLA. Alonzo's weight, length, and head circumference were normal. Small blisters were found on his palms and soles and he had yellow discharge from his eyes consistent with bacterial conjunctivitis. No signs of a middle ear infection were detected, and the physical examination of his heart and lungs was normal. The liver and spleen were not enlarged. Alonzo was admitted to the hospital. Blood tests showed that his hemoglobin was 8.4g/dL (low), his platelet count was 460,000 (slightly elevated), and his white blood cell count was 8000/l (normal), of which 56% were eosinophils (normal <5%), 23% monocytes (normal 10%), 15% neutrophils, and 6% lymphocytes (normal 50%). Alonzo's serum IgG level was 55mg/dL (normal 400mg / dL ), IgA and IgM were undetectable, and IgE was 7200IU/ml (normal <50IU/mL ). A skin biopsy showed that the dermis was infiltrated with large numbers of eosinophils, lymphocytes, and macrophages. An Xray of Alonzo's chest showed clear lungs and a normal cardiac shadow; there was no thymic shadow. After admission, Alonzo developed severe infections with pus from which Staphylococcus aureus and Candida albicans were cultured and he also developed a thrush with Candida albicans.