Epidemiology of pyoderma gangrenosum: Results from an Italian prospective multicentre study

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterised by painful, necrotic ulcerations. PG is described as a rare disease: the world‐wide incidence is estimated to be around 3 to 10 cases per million population per year. These estimations are based mostly on case reports and retrospective case series; there are no prospective, multicentre studies on the matter. The apparent rarity of PG is in contrast with our clinical perception as dermatologists: in our opinion, PG is not so uncommon. Therefore, we decide to investigate the epidemiology of PG in the Italian population and confirm our clinical suspicions that it is not an orphan disease. We enrolled all patients diagnosed with PG in 8 Italian Dermatological Departments from 1st October 2014 to 1st November 2015, and we recorded their features. Our data, collected from 64 patients, are in accordance with those of the published literature regarding the epidemiology and features of PG. In an Italian population of roughly 8 million inhabitants of 7 provinces, we found an incidence of 5.17 new cases per million population per year. Unlike our predictions before the study, we confirmed the world‐wide incidence of PG. To our knowledge, this is the first observational, multicentre study on PG. We hope that it provides a stimulus for further researches on PG and for the creation of an Italian register.     

坏疽性脓皮病伴溃疡性结肠炎继发肛瘘一例

坏疽性脓皮病伴溃疡性结肠炎继发肛瘘者少见,相关治疗方案亦少见报道,本文报告1例为坏疽性脓皮病 伴溃疡性结肠炎继发肛瘘,持续应用激素联合免疫抑制剂效果不佳,改用生物制剂后好转的诊治经过。     

Ulcerative Colitis

Ulcerative colitis (UC) is a chronic inflammatory bowel disease that can involve any aspect of the colon starting with mucosal inflammation in the rectum and extending proximally in a continuous fashion. Typical symptoms on presentation are bloody diarrhea, abdominal pain, fecal urgency, and tenesmus. In some patients, extraintestinal manifestations may predate the onset of gastrointestinal symptoms. A diagnosis of UC is made on the basis of presenting symptoms consistent with UC as well as endoscopic evidence showing continuous and diffuse colonic inflammation that starts in the rectum. Biopsies of the colon documenting chronic inflammation confirm the diagnosis of UC. Most cases are treated with pharmacological therapy to first induce remission and then to maintain a corticosteroid-free remission. There are multiple classes of drugs used to treat the disease. For mild to moderate UC, oral and rectal 5-aminosalycilates are typically used. In moderate to severe colitis, medication classes include thiopurines, biological agents targeting tumor necrosis factor and integrins, and the small-molecule Janus kinase inhibitors. However, in up to 15% of cases, patients in whom medical therapy fails or who have development of dysplasia secondary to their long-standing colitis will require surgical treatment. Finally, to minimize the complications of UC and adverse events from medications, a working collaboration between primary care physicians and gastroenterologists is necessary to make sure that vaccinations are optimized and that patients are screened for colon cancer, skin cancer, bone loss, depression, and other treatable and preventable complications.     

Association of pyoderma gangrenosum and sterile osteomyelitis in a patient having myelodysplastic syndrome with der(1;7)(q10;q10)

Neutrophilic dermatoses such as Sweet's disease and pyoderma gangrenosum (PG) are occasionally associated with myelodysplastic syndrome (MDS). We present here a 67-yr-old male having PG and sterile osteomyelitis in association with underlying MDS (refractory anemia) and Crohn's disease. To establish the diagnosis of MDS, sternal bone marrow puncture was performed, which showed chromosomal abnormality containing der(1;7)(q10;q10). After the puncture, he suffered from gradually progressive skin ulceration, flare, and bone pain. Magnetic resonance imaging (MRI) of the sternum showed severe inflammation in the sternum and the overlying subcutaneous tissue. All of the cultures obtained from the wound were negative for both bacteria and fungus. Biopsy was performed from the antero-sternal skin lesion, which showed epidermal ulceration with prominent infiltration of neutrophils. He was thus diagnosed as having PG and sterile osteomyelitis, and was treated with prednisolone, which completely resolved the symptoms. We consider that the bone marrow aspiration in the present patient provoked PG and sterile osteomyelitis. As was previously reported by others, certain chromosomal abnormalities in MDS may be related with the development of neutrophilic dermatoses.     

Management of Peristomal Pyoderma Gangrenosum

PURPOSE This study was designed to evaluate the presentation, management, and outcome of peristomal pyoderma gangrenosum at a specialist colorectal unit and develop a strategy for therapy.METHODS Patients with peristomal pyoderma gangrenosum were identified from a prospectively accrued Institutional Review Board-approved stoma database. Data were collected regarding demographics, disease status, history of illness, time to healing, and treatments used from the database and by chart review.RESULTS Sixteen patients presented between 1997 and 2002 with peristomal ulceration consistent with a diagnosis of peristomal pyoderma gangrenosum. Diagnosis was predominantly clinically based on a classic presentation of painful, undermined peristomal ulceration. The underlying diagnosis was Crohn’s disease in 11 patients, ulcerative colitis in 3, indeterminate colitis in 1, and posterior urethral valves in 1. At the time of development of peristomal pyoderma gangrenosum, the underlying disease was active in 69 percent of patients. Stoma care, ulcer debridement with unroofing of undermined edges, and intralesional corticosteroid injection was associated with a 40 percent complete response rate and further 40 percent partial response rate. Of five patients who received infliximab, four (80 percent) responded to therapy. Complete response after all forms of therapy, including stoma relocation in seven patients, was 87 percent.CONCLUSIONS Local wound management and enterostomal therapy are extremely important for patients with peristomal pyoderma gangrenosum. Infliximab may provide a useful option for those failing other forms of medical therapy. Relocation of the stoma is reserved for persistent ulceration failing other therapies, because peristomal pyoderma gangrenosum may recur at the new stoma site.Reprints are not available.     

Rosacea fulminans triggered by high-dose vitamins B6 and B12

Rosacea fulminans is a rare variant of rosacea conglobata that occurs almost exclusively in women well past adolescence. The aetiology is unknown, although immunological, hormonal, and vascular factors have been suggested. We report the case of a 17-year-old girl with rosacea fulminans that was temporally associated with daily ingestion of high-dose vitamin B supplements. The onset was sudden and cosmetically disabling. The eruption improved when the vitamin supplement was discontinued and a therapeutic regimen including isotretinoin and methylprednisolone was introduced. It seems appropriate to consider the possibility of such a vitamin B-triggered condition in cases of subjects presenting new or exacerbating facial eruptions.     

Concurrent pyoderma gangrenosum and infection with Scedosporium apiospermum

We present a patient with pyoderma gangrenosum (PG) following hip surgery, who developed an exacerbation of her ulceration in conjunction with new areas on her lower limbs. Clinically, features of PG and deep fungal infection were apparent. Scedosporium apiospermum was isolated from the ulcers.