[CIS PIDD] [cis-pidd] leukocyte adhesion def/pyoderma gangrenosum

[Karin Chen]

Dear All:

I would appreciate any suggestions on treatment of pyoderma gangrenosum-like wounds in the setting of leukocyte adhesion deficiency.

We are caring for a 9 year old girl with type I leukocyte adhesion deficiency, moderate phenotype.  She has developed a 2 month history of pyoderma gangrenosum-like lesions encompassing nearly the entire left arm, upper arm (this due to a prior central line site), and new lesions with any trauma (IV placement, blood draw needlesticks).  All cultures have been negative and pathology and gross view of lesions is consistent with pyoderma gangrenosum-like wounds.

Her care has included (in this order):
Twice weekly dressing changes under sedation/anesthesia -has developed horrible chronic pain at wound sites
Topical GM-CSF applied at time of dressing changes.
Prednisone 1mg/kg daily with slow taper
IVIG 2g/kg
cyclosporine twice daily with trough 100mcg/L
methylprednisolone pulse 30mg/kg x 1 (1gram dose)
repeat IVIG 1gram/kg
topical clobetasol to small lesion sites

Her inflammatory markers have fluctuated with response to immune modulators, now currently CRP quite high at 11.4 (normal <1.0). New lesions develop, and the old lesions have not progressed but are not decreasing size either.

Parents are now planning for hematopoietic stem cell transplant when we can get the lesions healed enough so that bleeding will not be an issue, but there is no end in sight.

I would appreciate any suggestions.

Best regards,
Karin

Karin Chen, MD
Assistant Professor
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
University of Utah
karin.chen at hsc.utah.edu




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