HYPERBARIC OXYGEN THERAPY FOR PYODERMA GANGRENOSUM

Hyperbaric oxygen administered systemically has been advocated in the treatment of pyoderma gangrenosum. Owing to the serious potential risks of oxygen toxicity with systemic administration, we have devised a simplified technique for the administration of hyperbaric oxygen topically, and have used this method successfully to treat two patients with pyoderma gangrenosum, in whom the underlying etiology was not apparent, and specific therapy, therefore, not possible. The abundant formation of granulation tissue and the ability of the hyperbaric oxygen to arrest further extension of the ulceration suggests that this form of therapy might become the treatment of choice for all ulcers of the pyoderma gangrenosum type.     

A case of cytomegalovirus colitis following immunosuppressive treatment for pyoderma gangrenosum

We report a case of pyoderma gangrenosum (PG) complicated by cytomegalovirus (CMV)-induced colitis. A 79-year-old woman with PG was treated with corticosteroid and cyclosporin. She had blood in her stool and advancing anemia during the treatment. A colonoscopic biopsy specimen from the colon revealed typical CMV-infected cells with CMV inclusions confirmed by immunohistochemistry. Furthermore, there were many CMV-antigen-positive leukocytes, suggesting an active CMV infection, which is serious in compromised hosts. Although ulcerative colitis and Crohn's disease are well known as complications of PG, CMV enterocolitis should be considered in the differential diagnosis of enterocolitis in immunocompromised patients.     

Post-surgical pyoderma gangrenosum in otherwise healthy patient with history of COVID-19

There are increasing reports of autoimmune and dermatologic sequelae of COVID-19. We describe an otherwise healthy patient with recent history of serious COVID-19 infection who developed post-surgical pyoderma gangrenosum following bilateral reduction mammoplasty and was successfully treated with infliximab, mycophenolic acid, and corticosteroids. We present this case to highlight the lingering systemic proinflammatory effects of COVID-19 infection that may increase the risk of rare autoimmune complications of surgery. As a complete understanding of the long-term effects of COVID-19 is poorly understood, patients with a history of COVID-19 infection should be appropriately counseled to these possible risks when discussing surgery.     

Pyoderma gangrenosum: an updated review

Pyoderma gangrenosum is a rare, ulcerative, cutaneous condition. First described in 1930, the pathogenesis of pyoderma gangrenosum remains unknown, but it is probably related to a hyperergic reaction. There are various clinical and histological variants of this disorder. Pyoderma gangrenosum often occurs in association with a systemic disease such as inflammatory bowel disease, rheumatologic disease, paraproteinaemia, or haematological malignancy. The diagnosis, mainly based on the clinical presentation and course, is confirmed through a process of elimination of other causes of cutaneous ulcers. Local treatment may be sufficient for mild disease, while for severe cases, systemic immunosuppressants are the mainstay. Long-term treatment with these agents is often required, but this can expose patients to adverse side-effects.

Conflicts of interest

None declared.     

Effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia

Introduction: Hyperzincaemia and hypercalprotectinaemia with systemic inflammation, recurrent infections, hepatosplenomegaly, arthritis, anemia, cutaneous inflammation, and failure to thrive is an extremely rare disease and no therapy is reported. Aim: To evaluated the effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia in terms of serum cytokine level changes before and after treatment. Methods: A 10-year-old girl was admitted suffering from pyoderma gangrenosum, hepatosplenomegaly, anemia that was unresponsive to iron supplementation, persistent inflammation, arthritis, and increased serum zinc. The level of serum calprotectin was extremely high; therefore, we diagnosed hyperzincaemia and hypercalprotectinaemia and started cyclosporine A treatment. Twelve cytokines in serum were measured before and one year after treatment. Results: Cyclosporine A was very effective. Her skin lesion and joint pain were alleviated and quality of life was markedly improved. C-reactive protein had decreased and anemia had improved. While zinc levels had fallen, calprotectin remained at an extremely high level. Of the cytokines examined, interleukin -6 serum levels had fallen and interleukin -8 showed a marked reduction after treatment.

Conclusion: Cyclosporine A is effective for hyperzincaemia and hypercalprotectinaemia. Serum interleukin -8 may be useful in assessing the therapeutic effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia.     

A case of chronic perianal pyoderma treated with the recycled skin graft method

We present here a case of chronic perianal pyoderma (CPP), who was treated with a novel skin graft. The patient was a 28-year-old male who had suffered from painful abscesses and nodules on his buttocks for over 10 years. Although the abscesses and nodules were initially restricted to one buttock, they gradually spread over both buttocks. He visited our hospital in July of 2000. He was clinically diagnosed as CPP and treated with oral doses of antibiotics. When admitted to our hospital in September of 2000, an anal fistula was also found. The operations for CPP and anal fistula were simultaneously performed. After the anal fistula treatment, the lesions of CPP were totally resected. In this procedure, we removed the epidermis and upper dermis from the excised CPP lesions and grafted them on the defects of the excised lesion. There has been no recurrence of the CPP. This operation procedure, which we call the "recycled skin graft method" is less invasive, because a donor site for the skin graft is unnecessary. We considered it to be as one of the effective treatments for CPP.     

A continuum of neutrophilic disease occurring in a patient with ulcerative colitis

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders, Aiypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological) dyscrasias but not. to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum,; Sweet's syndrome, erythema elevatum diutinum and. subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.     

Pyogenic arthritis,pyoderma gangrenosum,and acne syndrome in a Chinese family: A case report and review of literature

BACKGROUNDPyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is a rare autosomal dominant genetic disease characterized by severe autoimmune inflammation, caused by mutations in the PSTPIP1 gene. Due to PAPA heterogeneous clinical manifestation, misdiagnosis or delayed diagnoses are difficult to avoid. With the use of whole-exome sequencing, we identified a missense mutation in the PSTPIP1 gene in a Chinese family. To the best of our knowledge, this is the first case of PAPA reported in China.CASE SUMMARYA 9-year-old boy suffered from recurrent aseptic pyogenic arthritis triggered by minor trauma or few obvious predisposing causes for more than 3 years. Pyogenic arthritis occurred every 3-5 mo, affecting his knees, elbows, and ankle joints. Treatments, such as glucocorticoids, antibiotics, even surgeries could alleviate joints pain and swelling to some extent but could not inhibit the recurrence of arthritis. Similar symptoms were present in his younger brother but not in his parents. According to the whole-exome sequencing, a missense mutation in exon 11 of the PSTPIP1 gene (c.748G>C; p.E250Q) was detected in the boy, his younger brother and his father. Taking into account the similar phenotypic features with PAPA syndrome reported previously, we confirmed a diagnosis of PAPA syndrome for the family.CONCLUSIONIn this case, a missense mutation (c.748G>C; p.E250Q) in PSTPIP1 gene was identified in a Chinese family with PAPA syndrome. Previous studies emphasize the fact that PAPA syndrome is hard to diagnose just through the clinical manifestations owing to its heterogeneous expression. Genetic testing is an effectual auxiliary diagnostic method, especially in the early stages of pyogenic arthritis. Only if we have a deep understanding and rich experience of this rare disease can we make a prompt diagnosis, develop the best clinical treatment plan, and give good fertility guidance.     

Investigation of new co‐factors in 49 patients with pyoderma gangrenosum

Background: Pyoderma gangrenosum is a rare, destructive, ulcerative neutrophilic dermatosis of unknown origin that has been investigated insufficiently in clinical studies. According to current textbooks, it is often associated with chronic inflammatory bowel diseases or other autoimmune disorders. Patients and methods: We retrospectively analyzed data from 49 patients with pyoderma gangrenosum. Results: Our results showed that although only 6.1 % of patients had chronic inflammatory bowel disease, 22.4 % patients had a malignancy, 18.4 % had chronic renal insufficiency, and 42.8 % had anemia. A potentially relevant aspect that has received little attention is an association with endocrine diseases in 38.8 % of patients. 28.6 % of patients had diabetes. Given that 32.6 % of patients were obese, a potential association with metabolic syndrome may be considered as a possible new risk factor for pyoderma gangrenosum. Conclusions: The clinical data from the present study are insufficient for drawing any firm conclusions. We did, however, observe an association between pyoderma gangrenosum and certain as yet unreported co‐factors, in particular metabolic syndrome. This should be considered in further studies.     

Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathy

A 46-year-old man with a 3-year history of pyoderma gangrenosum was admitted with ulceration (6 x 5 cm), on the right leg. Previously he had been treated with tapering doses of prednisone (maximum dose 1 mg/kg per day); however, he had had a few exacerbations following each taper of prednisone dose. Immunoelectrophoresis demonstrated monoclonal IgA gammopathy of lambda light chains. Abdominal echography and abdominal computed tomographic scan revealed multiple splenic abscesses. Treatment was started with oral prednisone (1 mg/kg per day) and cyclosporin (5 mg/kg per day) and 6 weeks later complete remission was achieved. Systemic involvement in pyoderma gangrenosum is very rare, and according to our knowledge there are only a few cases with spleen involvement.