[CIS PIDD] [cis-pidd] LAD1 with pyoderma gangrenosum. Case for transplantation?

[Anders Fasth]

Dear All,
I need your advice. 
A 26 years old male is referred to me because of uncontrolled pyoderma gangrenosum (PG). He had onset of his PG at 3 years of age, no infectious symptoms. Initially, his PG was quite well controlled for the first 10 years, but not for the last 10 years. He has tried everything -  steroids, azatioprin, metotrexate, different kinds of TNF blockers, Adacolumn, IVIG etc. Right now he is treated with prednison, MMF and Cimzia with poor result. (case report in Acta Derm Venereol 2015; 95: 349–351)

The interesting finding is that he has LAD1, this was revealed about 6 years ago. Heterozygous mutations.  There is a recent publication in Clin Imm with 4 more cases of LAD1 who’s only manifestation is PG. Our patient has no other symptoms or signs but PG.  Lymphocyte populations normal, immunoglobulins  and specific antibodies too. 

As we cannot control his PG the question of HSCT has come up. Those against say that his PG might be worsen after transplantation. But I do not find anything in the literature that supports that fear. I can find a case report - HSCT for  Crohn and severe PG - where the PG healed completely after transplantation 

Anyone that has  the experience of transplanting a patient with pyoderma? Or opinions against or for transplantation?

Your input is highly appreciated 

Best

Anders

Anders Fasth, MD, PhD
Professor of Pediatric Immunology, 
Dept of Pediatrics, University of Gothenburg
Address: The Queen Silvia Children’s Hospital,
SE-416 85 Göteborg, Sweden
Tel +46-31-343 5220 (343 4000 switchboard)
Mobile +46-70-0852717 (work)
Mobile +46-70-687 5970 (private) 
Fax +46-31-707 0694

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