[PAGID] Eosinophilic abscesses in a CGD patient

[Berger, Melvin]

Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Dewton Vasconcelos
Sent: Wed 6/2/2010 1:06 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Eosinophilic abscesses in a CGD patient


Dear Howard, good afternoon

Very interesting case. We have seen some patients with deep mycosis (histoplasmosis and paracoccidioidomycosis) presenting eosinophilic granulomas. These patients presented very severe widespread disease, with elevated IgE and eosinophilia in the tissues and peripheral blood. As these diseases are intracellular and intravesicular such as are the mycobacteriosis, I think that we should think in the possibility.
Nevertheless, none of these patients have been diagnosed as presenting a PID.
I would also think about the possibility of a parasite (at least in Brazil) - has this patient traveled to any place that might be a source of any unapparent infection or infestation? Other possibility might be a mild form of Langerhans cell histiocytosis (eosinophilic granuloma). Certainly drug hypersensitivity is a possible differential diagnosis too.
What about the possibility of another disease such as the hyper IgE complex (STAT-3 or DOCK-8) instead of CGD?
Nevertheless I think that a strong reactor to PPD in the US is very suggestive of tuberculosis and I would treat for Tbc simultaneously to the laboratorial investigation and look for complications of the eosinophilia (he is nowadays with 1800 eosino/ml) but if the counts rise I would think in treating him with corticosteroids.
As far as I know there is only one reference on CGD and eosinophilic granulomas due to Aspergillus infection (Eur Respir J 1997; 10: 2166-2170).

All the best,

Dewton

Dewton de Moraes Vasconcelos, M.D.; Ph.D.
University of São Paulo Medical School



Howard Lederman 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 <mailto:Hlederm1 at jhem.jhmi.edu>  
	
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