[PAGID] Eosinophilic abscesses in a CGD patient

[Yesim Yilmaz]

Dr.Lederman,
have you considered quantiferon gold study?
could this be a severe form of EE? (still it doesn't explain the positive
PPD).
Yesim

Yesim Yilmaz Demirdag, MD
Section of Allergy & Immunology
West Virginia University School of Medicine
Morgantown, WV
ydemirdag at hsc.wvu.edu

On Wed, Jun 2, 2010 at 12:29 PM, Howard Lederman <hlederm1 at jhmi.edu> wrote:


>  Colleagues,

>

>

>

> I have a 4 ½ year old male patient who was diagnosed with chronic

> granulomatous disease at 5 weeks of age after presentation with bilateral

> neck masses (cervical lymphadenitis caused by methicillin-sensitive S.

> aureus).  He has had multiple episodes of cervical lymphadenitis, requiring

> parenteral antibiotics.   He is maintained on interferon gamma (3x/week) and

> Ciprofloxacin (TMP/SMZ caused leukopenia and thrombocytopenia).  Other

> medications are iron and multivitamin daily.

>

>

>

> He has a history of bloody stools in early infancy thought to be due to

> cow’s milk enterocolitis.  Symptoms resolved when he was switched to an

> elemental formula, and a colonoscopy while on Elecare was normal.  He has

> introduced to cow’s milk at 12 months of age without problems.

>

>

>

> When he was 3 ½ yrs old, he had bloody stools and poor weight gain.

> Cultures, and stool test for ova and parasites were normal. RASTs to food

> allergens were negative.   Milk elimination did not help.  In Jan 2010, EGD

> showed chronic duodenitis, and colonoscopy showed many areas with crypt

> distortion, prominent lamina propria eosinophils and rare intraepithelial

> eosinophils.  Granulomas were *not *seen.  He was started on Apriso (mesalamine)

> in early April to treat inflammatory bowel disease.

>

>

>

> At the end of April, he developed fevers to 103 F with no obvious source.

> Over a two week period, his WBC increased to WBC 30,000 with 44% polys, 43%

> lymphs and 6% eos.  CRP 16 mg/dL.  He then developed abdominal distension

> and a new hydrocele over a short period of time, due to bulky

> lymphadenopathy involving the mesentery and retroperitoneum.  A PPD was

> placed and was positive with 15 mm induration.

>

>

>

> He underwent an excisional biopsy of several mesenteric nodes so that we

> could identify the mycobacterium that we were confident was the cause of his

> acute symptoms. No acid fast organisms were seen in the lymph nodes.

> Cultures are still negative but it has been only 2 weeks so far.  However,

> the lymph node histology was a complete surprise to us.  There were

> granulomas, but the center of many of them were filled with abscesses of *

> eosinophils*!      [The CIS has posted a photomicrograph of the lymph node

> biopsy  at http://www.clinimmsoc.org/UserFiles/file/pagid_lederman.pdf]

>

>

>

> Our pathologists favor a diagnosis of Churg-Strauss based only upon the

> histology.   The Infectious Diseases  group thinks that this represents a

> drug hypersensitivity reaction and wants us to stop Apriso.  Neither

> diagnosis explains the positive PPD.

>

>

>

> Does anyone have a differential diagnosis for this?

>

>

>

> Howard

>

> Howard M. Lederman, M.D., Ph.D.

> Professor of Pediatrics, Medicine and Pathology

> Division of Pediatric Allergy and Immunology

> Johns Hopkins Hospital - CMSC 1102

> 600 N. Wolfe Street

> Baltimore, MD 21287-3923

> Phone: 410-955-5883

> Fax: 410-955-0229

> Email: Hlederm1 at jhmi.edu <Hlederm1 at jhem.jhmi.edu>

>

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>

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