[CIS-PAGID] Recalcitrant buttock ulcer in a 2yo with X-linked CGD

[Turvey, Stuart]

Dear Colleagues,

I would value your input on a patient here in Vancouver, Canada.

We recently diagnosed a 2yo boy with X-linked chronic granulomatous disease (CGD) and we are moving toward HSCT.

Unfortunately, we have had trouble clearing up a lesion on his left buttock. Approximately 6 months ago this lesion began as a small ‘boil’ which ultimately broke down and drained pus. We are now left with a ~1.5cm diameter ulcer with a slightly rolled edge, base with granulation tissue and minimal surrounding erythema (see attached image).

The unsuccessful steps we have taken to heal this lesion have included:
i) Biopsy with extensive culturing and histological examination: no organisms identified
ii) 10 days empiric antimicrobial therapy with meropenem, vancomycin and caspofungin: no change
iii) 14 days prednisone (1mg/kg/day divided BID): no change
iv) Careful wound care and dressings

What should we do next? We are considering topical GM-CSF, but no one here has any experience with this approach.

We really want to get this lesion cleared up before transplantation.

I thank you in advance for your input.

Stuart



Stuart Turvey MB BS DPhil
Associate Professor
Division of Infectious and Immunological Diseases
University of British Columbia
BC Children's Hospital and Child & Family Research Institute
950 West 28 Avenue
Vancouver BC V5Z 4H4
Ph: 604 875 2345 x5094
Fax: 604 875 2226
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