Pyoderma Gangrenosum Associated with Sclerosing Cholangitis, Type 1 Diabetes Mellitus and Ulcerative Colitis

We describe the case of a 22-year-old black female with type 1 diabetes mellitus diagnosed when she was 12 years old. She first presented (March 1994) with pustules and ulcerations on the upper and lower limbs, trunk and scalp at the age 17. The diagnosis of pyoderma gangrenosum was made. Since presentation, changes in liver function were detected and subsequent study led to the diagnosis of sclerosing cholangitis. The diagnosis of ulcerative colitis was made after colonoscopy. Partial response was obtained with minocycline and clofazimine, but treatment with 5-aminosalicylic acid achieved no improvement of the ulcerations. Liver transplantation, followed by immunosuppressive therapy led to complete regression of the cutaneous lesions.

Pyoderma gangrenosum and ulcerative colitis in the tropics

Pyoderma gangrenosum is a rare inflammatory skin condition, characterized by progressive and recurrent skin ulceration. There may be rapidly enlarging, painful ulcers with undermined edges and a necrotic, hemorrhagic base. Disorders classically associated with pyoderma gangrenosum include rheumatoid arthritis, inflammatory bowel disease, paraproteinemia and myeloproliferative disorders. There have been some reports of the occurrence of pyoderma gangrenosum in Africa, and in Nigeria, but only one specifically reported pyoderma gangrenosum in association with ulcerative colitis. We report on a 45-year-old man who presented with pyoderma gangrenosum associated with ulcerative colitis; the second report in Nigeria. The skin lesions were managed with daily honey wound dressings. Oral dapsone and prednisolone were started. The frequency of the bloody diarrhea decreased, and was completely resolved by the second week after admission. The ulcers also showed accelerated healing. The goal of therapy is directed towards the associated systemic disorder, if present.

Pyoderma gangrenosum after minor trauma in a pregnant woman, mistaken for necrotizing fasciitis: report of a case and literature review.

Abstract Background: Pyoderma gangrenosum is an ulcerative, non-infectious skin disorder. However, it can be mistaken as necrotizing fasciitis, a life-threatening infective condition. We describe here a case of pyoderma gangrenosum after minor trauma treated as necrotizing fasciitis. METHODS: Case report and literature review. CASE REPORT: A 27-year-old pregnant nurse had a pretibial wound after a fall on a rough surface. When erythema developed and no response to empirical antibiotic therapy was observed, multiple debridements were performed. Paradoxically, her condition became worse. The diagnosis of pyoderma gangrenosum was suspected. Treatment with corticosteroids was started and this was successful. CONCLUSION: Pyoderma gangrenosum can mimic infectious necrotizing fasciitis. Differentiating these two conditions is important because mistreatment of pyoderma can lead to disfigurement.

Pyoderma gangrenosum: défi diagnostique et thérapeutique [Pyoderma gangrenosum: diagnostic and therapeutic challenge]

Pyoderma gangrenosum is a rare ulcerative disease associated with inflammatory bowel disease, arthritis or haematological malignancies. The diagnosis of Pyoderma gangrenosum is often delayed while consideration is given to the more likely diagnoses of wound breakdown or bacterial infection. The outcome depends on early diagnosis and on excellent collaboration between the surgical team and the infectious disease specialist. We present two puzzling cases of Pyoderma gangrenosum and discuss the physiopathology, the diagnosis strategy and the management. Le Pyoderma gangrenosum constitue une entité clinique méconnue, se présentant classiquement par des ulcérations cutanées inflammatoires et douloureuses. Le contexte clinique retrouve fréquemment une pathologie inflammatoire digestive, une néoplasie ou une atteinte rhumatologique concomitante. Le diagnostic est trop souvent tardif, après de multiples traitements antibiotiques et chirurgicaux, et nécessite une excellente coordination entre le médecin traitant, les équipes chirurgicales et les spécialistes des maladies infectieuses. Au travers de deux cas spectaculaires, nous rappelons quelques notions de base sur la physiopathologie, la présentation clinique et la prise en charge du Pyoderma gangrenosum.

Le pyoderma gangrenosum: pathologie rare ou diagnostic omis [Pyoderma gangrenosum: a rare pathology or an omitted diagnosis?]

Pyoderma gangrenosum is a rare pathology of uncertain etiology. It is an inflammatory dermatosis that causes cutaneous necrosis with a characteristically rapid evolution. No precise diagnosis criteria has been established and pyoderma gangrenosum remains an exclusion diagnosis. In 40 to 50% of the cases, the evolution of pyoderma gangrenosum can be worsened by a nonspecific external stimulus such as a trauma or a surgery. This phenomenon, called pathergy, conduces to avoid any surgical excision of the lesion. However, the diagnosis is often omitted and the rapid evolution of the cutaneous necrosis forces the surgeon to perform a surgical debridement of the wound. It causes a pejoration of the lesion. It can have disastrous consequences. We present here three different case reports.

Aesthetic Breast Augmentation Mastopexy Followed by Post-surgical Pyoderma Gangrenosum (PSPG): Clinic, Treatment, and Review of the Literature.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare autoinflammatory neutrophilic ulcerative skin disease, often developing after a trauma or surgical wounds. In the literature there are several reports of post-surgical PG (PSPG) of the breast. The authors of this article experienced an impressive case of PSPG after an aesthetic breast augmentation mastopexy. PSPG is a rare but severe complication in this elective aesthetic surgical procedure. METHOD: A systematic review of the literature was performed, focusing on PSPG after aesthetic breast surgery (augmentation mammoplasty/mastopexy). The online databases Pubmed, Medline, and Cochrane were used and additionally a Google© search was conducted. We compared the data obtained from a systematic literature review to an index case of PSPG after esthetic augmentation mammoplasty. RESULTS: The literature search identified seven articles describing eight cases of PSPG after aesthetic breast surgery. In four of these cases augmentation mammoplasty had been carried out, in two cases mastopexy and in two cases augmentation mammoplasty and mastopexy (augmentation mastopexy). The patient we treated and describe in this paper underwent an augmentation mastopexy outside our clinic. Eight patients suffered from local disease, at the site of surgical wounds, one patient had disseminated disease. Leukocytosis was present in five cases (out of nine). Eight patients had received corticosteroid treatment, one patient refused such treatment. The duration of corticosteroid treatment was on average for 41 days (range 21-60 days). In all cases, the areola had been spared. Complete healing of PSPG was observed on average after 5 months (range 1.5 months-1 year). DISCUSSION: PSPG of the breast after aesthetic breast surgery is rare, but every plastic surgeon should consider this possibility, especially if skin disease develops post-surgery, mimicking wound infection that does not respond to broad-spectrum antibiotic treatment. CONCLUSION: Although the literature does not recommend this step, implant removal is recommended by the authors because bacterial wound infection normally cannot be ruled out definitely in the early stages of disease. Additional surgical intervention should be limited to the absolute necessary and performed only under adequate systemic immunosuppressive therapy. LEVEL OF EVIDENCE V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Pyostomatitis vegetans and its relation to inflammatory bowel disease, pyoderma gangrenosum, pyodermatitis vegetans, and pemphigus

J Oral Pathol Med (2012) 41: 584588 Pyostomatitis vegetans (PSV) is an intraoral pustular eruption considered by most authors to represent the mucous analogous of cutaneous pyoderma gangrenosum and its vegetating presentations (pyodermatitis vegetans). A strong correlation of PSV with inflammatory bowel disease (IBD) is well documented. The histopathology of PSV lesions usually reveals acanthosis, and neutrophils and/or eosinophils infiltration with intraepithelial or subepithelial abscesses; acantholysis is present in some cases. We studied four patients with IBD that presented oral lesions suggestive of PSV. Two male and two female patients were included. The histopathology of oral lesions of two patients revealed findings typical for PSV. The other two patients showed findings typical for pemphigus vulgaris (PV), although the course of their symptoms paralleled that of the bowel disease. Our findings may suggest that pustular lesions in patients with IBD can be a presentation of both PSV and PV; adequate diagnosis is required because clinical presentation is very similar.

Wegener's granulomatosis presenting as pyoderma gangrenosum

Half of the patients with Wegener's granulomatosis develop skin lesions due to the systemic vasculitis. Wegener's granulomatosis should be included in the differential diagnostic considerations for necrotic ulcers, including leg ulcers. We present a case which demonstrates the importance of histological evaluation of a skin biopsy from the margin of the ulcer for establishing the diagnosis. Antineutrophil cytoplasmic antibodies with antigen specificity for proteinase 3 (PR3-ANCA) were detected supporting the diagnosis of Wegener granulomatosis. Further evaluation showed involvement of the eyes and kidneys. The ulcer rapidly healed under treatment with cyclophosphamide and corticosteroids.

Canakinumab in adults with steroid-refractory pyoderma gangrenosum

BACKGROUND Pyoderma gangrenosum (PG) is a rare, neutrophilic, ulcerative skin disease that is difficult to treat, especially when unresponsive to steroids. OBJECTIVES To determine whether canakinumab is an effective and safe treatment in PG. METHODS Five adult patients with clinically and histologically confirmed steroid-refractory PG were enrolled in this prospective open-label study. They received canakinumab 150 mg subcutaneously at week 0 with an optional 150 mg at week 2 in case of an inadequate response [Physician's Global Assessment (PGA) ≥ 2], and an optional 150-300 mg at week 8 depending on PGA. The primary clinical end point was clinical improvement (PGA at least -1 from baseline) and/or complete remission (PGA 0 or 1) at week 16. Real-time quantitative polymerase chain reaction was performed on skin samples to quantify cytokine mRNA levels. RESULTS Interleukin (IL)-1β and its known target genes IL6, CXCL8 and IL36A were significantly increased in lesional skin of PG. Under canakinumab therapy, four of five patients showed a decrease in target-lesion size, PGA and Dermatology Life Quality Index (DLQI), and three of five achieved complete remission. The mean diameter of target lesions decreased from 4·32 ± 2·6 cm at visit 1 to 0·78 ± 1·3 cm at visit 7 (P = 0·03). Mean DLQI decreased from 15 ± 5 at visit 1 to 8 ± 4 by visit 7 (P = 0·01). Adverse effects were reported in two patients: fatigue in one and worsening of disease at a nontarget lesion in the other. CONCLUSIONS Our data indicate that IL-1β plays a key pathogenic role in PG and canakinumab may represent a therapeutic option for steroid-refractory PG.

Frequency and risk factors for extraintestinal manifestations in the Swiss inflammatory bowel disease cohort.

OBJECTIVES: Data on the frequency of extraintestinal manifestations (EIMs) in Crohn's disease (CD) and ulcerative colitis (UC) and analyses of their risk factors are scarce. We evaluated their prevalence and risk factors in a large nationwide cohort of inflammatory bowel disease (IBD) patients. METHODS: IBD patients from an adult clinical cohort in Switzerland (Swiss IBD cohort study) were prospectively included. Data from validated physician enrolment questionnaires were analyzed. RESULTS: A total of 950 patients were included, 580 (61%) with CD (mean age 41 years) and 370 (39%) with UC (mean age 42 years). Of these, 249 (43%) of CD and 113 (31%) of UC patients had one to five EIMs. The following EIMs were found: arthritis (CD 33%, UC 21%), aphthous stomatitis (CD 10%, UC 4%), uveitis (CD 6%, UC 4%), erythema nodosum (CD 6%, UC 3%), ankylosing spondylitis (CD 6%, UC 2%), psoriasis (CD 2%, UC 1%), pyoderma gangrenosum (CD and UC each 2%), and primary sclerosing cholangitis (CD 1%, UC 4%). Multiple logistic regression identified the following risk factors for ongoing EIM in CD: active disease (odds ratio (OR)=1.95, 95% confidence interval (CI)=1.17-3.23, P=0.01), and positive IBD family history (OR=1.77, 95% CI=1.07-2.92, P=0.025). No risk factors were identified in UC patients. CONCLUSIONS: EIMs are a frequent problem in CD and UC patients. Active disease and positive IBD family history are associated with ongoing EIM in CD patients. Identification of EIM prevalence and associated risk factors may result in increased awareness for this problem and thereby facilitating their diagnosis and therapeutic management.

Treatment of extraintestinal manifestations in inflammatory bowel disease.

Inflammatory bowel disease (IBD) is a systemic disease associated with a large number of extraintestinal manifestations (EIM). EIM are present in 15-20% of patients with ulcerative colitis and in 20-40% of patients with Crohn's disease. The management of EIM is best provided by a multidisciplinary team, which includes specialists in the affected organ systems with training in the treatment of IBD. Therapeutic strategy is often empirical. This is explained by the paucity of randomized-controlled studies for the specific treatment of EIM in IBD and by the fact that treatment models are based on extrapolation from patients with similar conditions but without IBD. For most EIM, the mainstay of therapy is the treatment of the underlying active IBD. However, some EIM such as axial arthritis, pyoderma gangrenosum, uveitis and primary sclerosing cholangitis run a clinical course independent of IBD activity and need specific therapy (e.g. TNF antagonists in ankylosing spondylitis and skin manifestations). This review summarizes the conventional and novel (e.g. anti-TNF) treatment modalities, and the therapeutic implications for the management of extraintestinal symptoms in IBD, in order to assist clinicians in optimizing treatment strategies for IBD patients with EIM.

Chronology of extraintestinal manifestations relative to IBD diagnosis in the Swiss IBD Cohort

Background and Aims: Due to a p aucity of s uch data we aimed to a ssess the type and f requency o f extraintestinal manifestations ( EIM) in I BD p atients and to e valuate their chronologic behavior. Methods: A nalysis of d ata from t he Swiss Inflammatory Bowel Disease Cohort (SIBDCS) which c ollects data since 2 005 on a large sample o f IBD patients f rom hospitals and private practices across Switzerland. Results: A t total o f 1,143 patients were a nalyzed ( 572 (50%) female, mean age 42.1 ± 14.4 years), 629 with Crohn's disease (CD), 501 with ulcerative colitis (UC), and 13 with indeterminate colitis ( IC). Of t hese, 3 74 (32.7%) presented o ne to five E IM (65% w ith CD, 3 3% w ith UC, 2% w ith IC). O f those patients suffering from EIM, 4 1.7% p resented two, 1 2.4% t hree, 5 .3% four, and 3.2% f ive E IM d uring lifetime. T he initial EIM presented with the following frequencies: p eripheral a rthritis (PA) 6 3.4%, ankylosing spondylitis (AS) 8 .1%, primary sclerosing cholangitis (PSC) 6%, uveitis 5.7%, oral a phthosis 5.7%, erythema nodosum (EN) 5 %, other 3 .6%, pyoderma gangrenosum 1.8%, psoriasis 0.7%. In only 7.1% of cases, the EIM m anifested before IBD diagnosis was made (median time 28 months b efore IBD diagnosis, I QR 7 -60 months), in 9 2.9% EIM m anifested a fter e stablished IBD d iagnosis (median 72 months, IQR 9-147 months). Conclusions: EIM are a frequent problem in IBD patients. The vast m ajority of E IM m anifest a fter I BD d iagnosis has b een established. P eripheral a rthritis, a nkylosing spondylitis, a nd PSC represent the most frequent first manifestations of an EIM.