Masashi Ohno ¹, Shigeki Koyama, Mariko Ohara, Kazumi Shimamoto, Yu Kobayashi, Fumiyasu Nakamura, Kazuki Mitsuru, Akira Andoh

Intern Med.  2016;55(1):25-30. doi: 10.2169/internalmedicine.55.5422. Epub 2016 Jan 1.

A 36-year-old woman was admitted to our hospital due to swelling and redness of the left lateral malleolus and dorsum of the left foot with severe pain, with a flare-up of ulcerative colitis (UC). A pathologic examination by skin biopsy led to a diagnosis of pyoderma gangrenosum (PG). She was treated with the intravenous administration of prednisolone (60 mg/day), and granulocyte and monocyte adsorption apheresis (GMA) was performed twice-a-week for 5 weeks. This treatment dramatically improved both the skin and colonic mucosal lesions. These results suggest that a combination of GMA and corticosteroids might be recommendable to induce the remission of serious PG complicated with UC.

https://pubmed.ncbi.nlm.nih.gov/26726081/

https://www.jstage.jst.go.jp/article/internalmedicine/55/1/55_55.5422/_article

Uwe Wollina, Orphanet Journal of Rare Diseases volume 2, Article number: 19 (2007)

Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis. Clinically it starts with sterile pustules that rapidly progress and turn into painful ulcers of variable depth and size with undermined violaceous borders. The legs are most commonly affected but other parts of the skin and mucous membranes may also be involved. Course can be mild or malignant, chronic or relapsing with remarkable morbidity. In many cases PG is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatic or haematological disease and malignancy. Diagnosis of PG is based on history of an underlying disease, typical clinical presentation, histopathology, and exclusion of other diseases that would lead to a similar appearance. The peak of incidence occurs between the ages of 20 to 50 years with women being more often affected than men. Aetiology has not been clearly determined yet.

Pyoderma gangrenosum – a review | Orphanet Journal of Rare Diseases | Full Text (biomedcentral.com)

Keiko Okuma,Kouichi Mitsuishi,Toshio Hasegawa,Hitoshi Tsuchihashi,Hideoki Ogawa,Shigaku Ikeda,

https://doi.org/10.1111/j.1744-9987.2007.00498.x

Abstract: Granulocytapheresis (GCAP) therapy is a newly developed therapeutic modality for inflammatory bowel diseases such as ulcerative colitis and Crohn’s disease. Pyoderma gangrenosum (PG) is a chronic inflammatory skin disease characterized by the appearance of erythematous macules and plaques with pustules or nodules that rapidly progress to ragged, undermined multiple ulcers. We attempted GCAP therapy in a patient with PG resistant to prednisolone and various other immunosuppressants. GCAP therapy was initiated at three- to four-day intervals and a good response from all skin lesions, with eventual total epithelialization, was observed after 10 sessions of this therapy. Furthermore, circulating levels of inflammatory cytokines such as interleukin-8 (IL-8) and granulocyte colony stimulating factor (G-CSF) also decreased after the GCAP therapy. Our results suggest that GCAP is a safe and useful tool for the treatment of intractable PG, and that IL-8 and G-CSF are likely to be involved in the pathogenesis of PG.

https://onlinelibrary.wiley.com/doi/10.1111/j.1744-9987.2007.00498.x

Mariko Seishima ¹, Yoko Mizutani, Yoshinao Shibuya, Chikako Nagasawa, Takahiko Aoki

Ther Apher Dial 2007 Jun;11(3):177-82. doi: 10.1111/j.1744-9987.2007.00424.x.

Pyoderma gangrenosum presents with chronic skin ulcers and is histologically characterized by neutrophil infiltration throughout the dermis. It is also occasionally associated with ulcerative colitis, a type of inflammatory bowel disease, against which granulocyte and monocyte adsorption apheresis (GCAP) has recently shown remarkable efficacy. We performed GCAP on three refractory cases of pyoderma gangrenosum with painful bilateral leg ulcers and hereby report the results obtained. Patient 1 was a 43-year-old woman with a four-year history of recurrent painful skin ulcers treated with prednisolone and cyclosporine. Patient 2 was a 29-year-old woman who had been suffering from pyoderma gangrenosum with severe pain for two weeks, associated with an 11-year history of ulcerative colitis treated with prednisolone and salazosulfapyridine. Patient 3 was a 63-year-old man with a three-year history of recurrent ulcers with pain, suffering from rheumatoid arthritis treated with prednisolone and cyclophosphamide. The sizes of the lesions were reduced in all three patients following a weekly GCAP treatment for 10 or 11 consecutive weeks, and the re-epithelialization of ulcers were additionally observed in two patients. The pain disappeared dramatically in all three patients following two sessions of GCAP therapy. No adverse effects were observed for up to at least eight months after treatment. We therefore considered GCAP as one effective alternative to currently existing therapies, with regards to refractory cases of pyoderma gangrenosum.

https://pubmed.ncbi.nlm.nih.gov/17497998/

Takuro Kanekura,Koichi Kawahara,Ikuro Maruyama,Tamotsu Kanzaki Ther Apher Dial 2005 Aug;9(4):292-6. doi: 10.1111/j.1744-9987.2005.00284.x.

Pyoderma gangrenosum is an intractable skin disorder characterized by the development of erythematous pustules or nodules that rapidly progress to destructive, necrotizing, non-infective ulcers. We assessed the efficacy of granulocyte and monocyte adsorption apheresis (GCAP) therapy in two new patients, a 67-year-old man with ulcerations on his lower leg, and a 44-year-old man with turgid erythematous lesions with burrowing abscesses and sinus formation on his hip, groin, and thighs. Both patients received 10 GCAP treatments at 5-day intervals. Their skin lesions responded well. The 9 cmx6 cm ulcer on the lower right leg of the 67-year-old patient was completely covered by regenerated skin at the completion of therapy. The turgid skin lesions containing pustules and ulcers of the other patient showed amelioration and a marked decrease in the volume of exudate. Our results suggest that GCAP is a useful treatment modality for pyoderma gangrenosum.

https://pubmed.ncbi.nlm.nih.gov/16076369/

Ritsuko Yanaru-Fujisawa, Takayuki Matsumoto, Shotaro Nakamura, Shuji Kochi, Mitsuo Iida, Futoshi Kohda, Minako Hirahashi, Takashi Yao, Ryuichi Case Reports Inflamm Bowel Dis. 2005 Aug;11(8):780 DOI: 10.1097/01.mib.0000172558.39767.b7

Our case and the case of Kanekura et al⁸ suggest that circulating leukocytes may play an important role in the pathogenesis of PG and that GCAP in combination with corticosteroids may be a promising strategy for intractable PG. Furthermore, as has been the case for active UC, GCAP may be a choice for severe pouchitis. An accumulation of data with respect to the effect of GCAP on pouchitis seems to be warranted.

https://pubmed.ncbi.nlm.nih.gov/16043996/

https://academic.oup.com/ibdjournal/article/11/8/780/4685895

Ritsuko Yanaru-Fujisawa, Takayuki Matsumoto, Shotaro Nakamura, Shuji Kochi, Mitsuo Iida, Futoshi Kohda, Minako Hirahashi, Takashi Yao, Ryuichi Mibu Case Reports Inflamm Bowel Dis. 2005 Aug;11(8):780 DOI: 10.1097/01.mib.0000172558.39767.b7

https://pubmed.ncbi.nlm.nih.gov/16043996/