[PAGID] Eosinophilic abscesses in a CGD patient

[Howard Lederman]

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<mailto:Hlederm1 at jhem.jhmi.edu>

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