Atlas of Hematology

Radiographic Findings in Hyperimmunoglobulin E Syndrome
The aim of this study is to demonstrate the spectrum of radiologic findings of hyperimmunoglobulin E (hyper- IgE) syndrome that is relatively rare in adults. We describe the imaging findings of this rare disease in five patients. The study group consisted of 4 men and 1 woman. The final diagnosis was made according to laboratory findings in all cases. We detected lung involvement in 3 cases, paranasal sinus involvement in 4 cases and vascular involvement in 1 case. Soft tissues were involved in 5 cases. Bone involvement was demonstrated in 3 patients. The most frequent radiologic finding was widespread infectious involvement of soft tissues, bones and neighboring structures such as bursae and tendons and respiratory system.

Authors: Pınar Polat, Mecit Kantarcı, Fatih Alper, Serpil Erol, Refik Ali Sarı, Selami Suma, Zülal Özkurt

Keywords:

Year: 2002
Number: 4
Referance No: 93892

Figure 1a. A-15-year-old girl with hyper IgE syndrome. Axial CT section in width window shows a cavitary lesion due to Staphylococcus aureus infection at the upper apical segment of right lung.
Figure 1b. A-15-year-old girl with hyper IgE syndrome. On the lower section, multiple hematogenous focuses are demonstrated.
Figure 1c. A-15-year-old girl with hyper IgE syndrome. Axial CT section shows pneumothorax due to rupture of pneumatoceles to the pleural cavity during medication.
Figure 1d. A-15-year-old girl with hyper IgE syndrome. Axial contrast-enhanced CT section through the right knee joint shows thickening of the cutaneous and subcutaneous tissues and increased density of fatty tissue with streaky, irregular enhancement (arrows). Note the fluid collection in cutaneous and subcutaneous tissue with low density (asterisk).
Figure 2a. A-21-year-old man with the involvement of upper respiratory system and soft tissues. Coronal CT section of paranasal sinuses shows mucosal thickening of the bilateral maxillary and ethmoid sinuses
Figure 2b. A-21-year-old man with the involvement of upper respiratory system and soft tissues. Axial CT section through the elbow shows hypodense fluid collections in the cutaneous tissue (arrows) and abscess formation in the muscle (asterisk).
Figure 3a. A-27-year-old man with soft tissue, joint, bursae involvement. On T1-weighted coronal shoulder MR section, the cutaneous and subcutaneous tissues show increased thickness and decreased signal on the superior aspect of the joint (asterisk).
Figure 3b. A-27-year-old man with soft tissue, joint, bursae involvement. Coronal, contrast enhanced T1- weighted image shows contrast enhancement in the subcutaneous tissue. Note the hypointense abscess formation in subcutaneous tissue with peripheral enhancement (asterisk). Note the extending of infectious process to the acromioclavicular joint (arrowheads).
Figure 3c. A-27-year-old man with soft tissue, joint, bursae involvement. Coronal T2-weighted MR image shows high signal intensity in cutaneous, subcutaneous tissue (asterisk) and acromioclavicular joint. Note also hyperintense fluid in subacromial subdeltoid bursae.
Figure 4a. A-26-year-old man with lung, soft tissue, bone and tendon involvement. High resolution CT section shows bronchiectasis at the mediobasale segment of the right lung due to recurrent infections.
Figure 4b. A-26-year-old man with lung, soft tissue, bone and tendon involvement. Contrast-enhanced, axial T1-weighted MR image through the gluteus muscles shows hypodense mass lesions with ring enhancement at gluteus maximus muscles.
Figure 4c. A-26-year-old man with lung, soft tissue, bone and tendon involvement. Axial T2-weighted MR image of left ankle shows increased thickness and increased signal intensity in cutaneous and subcutaneous tissues. Note also thickening and increased signal intensity in the tendons of perenous brevis and longus due to spreading of infectious process neigbouring tissues (white arrow). Note also synovial cyst as increased signal intensity protruding posterior to the joint (asterisk).
Figure 4d. A-26-year-old man with lung, soft tissue, bone and tendon involvement. Axial CT section through the iliac wings shows contiguous spread of infectious process in the left iliacus muscle to the left iliac wing and note the formation of periostitis neighboring iliac wing (arrowheads). Contiguous spread of infection to sacrum is seen as a lytic lesion (asterisk). Left sacroiliac joint is seen as narrowed comparing right site and note also subchondral sclerosis due to infectious spread (curved arrow).
Figure 4e. A-26-year-old man with lung, soft tissue, bone and tendon involvement. Axial CT section through the upper margins of iliac wings shows periostitis (arrowheads) and hyperdense sequestreum formation (arrow) in the medulla.
Figure 5a. A-46-year-old man with soft tissue, bone, deep muscle and vessel involvement. Axial CT section shows bilateral hypodense psoas abscesses (arrows).
Figure 5b. A-46-year-old man with soft tissue, bone, deep muscle and vessel involvement. Color Doppler image shows typical swirling pattern of flow in the dorsal aspect of the dorsalis pedis artery. Spectral analysis shows to and fro flow in the neck of the aneurysm.

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