[PAGID] Eosinophilic abscesses in a CGD patient

[Vinh, Donald (NIH/NIAID [F]]

Given a febrile lymphadenopathy illness for the last 6 weeks or so with new-onset granulomas, a mycobacterial or filamentous fungal infection is a strong possibility. Because he is CGD, the mould infections to consider include:
(1) Hyalohyphomycetes, e.g. Aspergillus, Fusarium, Paecilomyces, Penicillium
(2) Dematiaceous - much less common, with only a few reports
(3) Mucoraceous - we think CGD patients are not at inherently increased risk, but the risk becomes apparent only after superimposed immunosuppression, which doesn't seem to be the case here.
(4) Oddball moulds
We don't think that CGD patients are at spontaneous increased risk for disseminated disease due to thermally-dimorphic endemic mycoses (e.g. cocci, histo).
Fungal stains may be helpful: GMS may be useful, but to look for (2), you could request a Fontana-masson stain to look for melaninized hyphae. Culture is obviously important, but you'd want to make sure the mycology lab doesn't discard "contaminants" (such as dematiaceous moulds or poorly-sporulating moulds).
Although Galactomannans are not helpful in CGD, I am unaware if Beta-glucan would be useful (could actually lead to some false+, so caution)

However, the PPD of 15 mm is quite alarming and wouldn't be accounted for by any of the fungi

Don


________________________________
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Howard Lederman
Sent: Wednesday, June 02, 2010 9:30 AM
To: pagid at list.clinimmsoc.org
Cc: SHOLLAND at niaid.nih.gov
Subject: [PAGID] Eosinophilic abscesses in a CGD patient

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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