Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum.

Pyoderma gangrenosum (PG) is an inflammatory condition characterized by chronic cutaneous ulcerations. There are three proposed PG diagnostic frameworks (Su, PARACELSUS, Delphi); however, they lack consensus, and their performance has not yet been validated in a well-defined cohort of patients with PG. In this cross-sectional retrospective cohort study, we sought to evaluate and compare the concordance of these diagnostic frameworks within a single-institution cohort of patients with PG. There were 47 patients from an initial 76 identified by International Classification of Diseases-9 and/or International Classification of Diseases-10 codes, where two PG experts agreed in their diagnosis of PG on the basis of clinical descriptions, photographs, and pathology. This group was the PG cohort by which we evaluated the performance and concordance of the diagnostic frameworks. The PARACELSUS score identified the highest proportion of patients with PG (89% [42 of 47 patients]), followed by Delphi and Su criteria, each at 74% (35 of 47 patients). Assessment of multirater agreement found that the three criteria agreed in their diagnoses for 72% of the patients (95% confidence interval = 60-85%); chance-adjusted agreement was determined to be 0.44 (95% confidence = 0.16-0.68, Fleiss' kappa). Future research should seek to refine these diagnostic frameworks and identify targeted methods of testing to reduce rates of PG misdiagnosis and patient misclassification in clinical trials.

Pyoderma Gangrenosum: A Critical Appraisal.

GENERAL PURPOSE: To provide information about pyoderma gangrenosum (PG), including pathophysiology, diagnostic criteria, and treatment. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant should be better able to: ABSTRACT: Pyoderma gangrenosum (PG) is an uncommon cutaneous disease, presenting with recurrent painful ulcerations most commonly on the lower extremities. The diagnosis is made according to a typical presentation, skin lesion morphology, skin biopsy, histopathology, and the exclusion of other etiologies. Classically, PG presents with painful ulcers with well-defined violaceous borders; other variants including bullous, pustular, and vegetative/granulomatous can also occur. Treatment of PG involves a combination of topical and systemic anti-inflammatory and immunosuppressive medications, wound care, antimicrobial agents for secondary infections, and treatment of the underlying etiology. This article is a continuing education review of the literature with a focus on the clinical application of the pathophysiology, diagnosis, and treatment of this challenging disease.

Ocular pyoderma gangrenosum: A systematic review.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare, ulcerative cutaneous disorder. Ophthalmic involvement in PG is atypical, but can have devastating consequences. OBJECTIVE: We sought to characterize ocular PG to allow for earlier diagnosis and therapy. To our knowledge, this is the first systematic review summarizing this clinical variant. METHODS: A systematic review was conducted using PubMed and Web of Science. Data were extracted and studies were qualitatively assessed and analyzed. RESULTS: We identified all 34 cases of PG involving the eye and periorbital area, and categorized them into 4 different subtypes. Common presenting signs include ulceration, peripheral ulcerative keratitis, and decreased visual acuity. Although it is often difficult to biopsy ocular PG, histologic features are nonspecific. Combined therapy using corticosteroids and further surgical reconstruction as needed is the mainstay of treatment. Cases of the eye/orbit in particular should be treated aggressively, as these are more likely to relapse compared with cases of the periorbital area. LIMITATIONS: Use of case reports, paucity of ocular PG cases, and heterogeneity of studies are limitations. CONCLUSION: PG should be considered in the differential diagnosis of ulceration of ocular/periocular tissues. An aggressive, early, multimodal treatment strategy should be used to prevent relapse, especially in cases of the eye/orbit.

A Review and Proposed Approach to the Neutrophilic Dermatoses of Childhood.

Neutrophilic dermatoses (NDs) are inflammatory skin conditions that are not associated with infection. The classification and clinical approach to these conditions in children is poorly described. This review classifies these conditions into five nosological subtypes: Sweet's syndrome, pyoderma gangrenosum, aseptic pustules, neutrophilic urticarial dermatoses, and Marshall's syndrome. In addition, we review the various secondary diseases that need to be excluded in the clinical management of the NDs of childhood, with a focus on the autoinflammatory conditions that the reader may not be familiar with. We propose a practical clinical approach to these disorders.

Pioderma gangrenoso / Pyoderma gangrenosum

El pioderma gangrenoso (PG) es una enfermedad de etiología desconocida, que se incluye en el grupo de las dermatosis neutrofílicas. Se reconocen cuatro variantes clínicas: ulcerativa,ampollar, pustulosa y vegetante. Puede presentarse en asociación con una enfermedad sistémica en aproximadamente el 50% de los casos. Para el diagnóstico es importantela exclusión de otras patologías, ya que no hay hallazgos específicos de laboratorio ni histopatológicos. Su manejo requiere tratamiento local y con frecuencia sistémico. Se presentan dos casos de PG ulcerosa...

Autoinflammatory skin disorders in inflammatory bowel diseases, pyoderma gangrenosum and Sweet's syndrome: a comprehensive review and disease classification criteria.

Pyoderma gangrenosum (PG) and Sweet's syndrome (SS) are skin diseases usually presenting with recurrent ulcers and erythematous plaques, respectively. The accumulation of neutrophils in the skin, characteristic of these conditions, led to coin the term of neutrophilic dermatoses to define them. Recently, neutrophilic dermatoses have been included in the group of autoinflammatory diseases, which classically comprises genetically determined forms due to mutations of genes regulating the innate immune response. Both PG and SS are frequently associated with inflammatory bowel diseases (IBDs); however, IBD patients develop PG in 1-3 % of cases, whereas SS is rarer. Clinically, PG presents with deep erythematous-to-violaceous painful ulcers with well-defined borders; bullous, pustular, and vegetative variants can also occur. SS is characterized by the abrupt onset of fever, peripheral neutrophilia, tender erythematous skin lesions, and a diffuse neutrophilic dermal infiltrate. It is also known as acute febrile neutrophilic dermatosis. Treatment of PG involves a combination of wound care, topical medications, antibiotics for secondary infections, and treatment of the underlying IBD. Topical therapies include corticosteroids and the calcineurin inhibitor tacrolimus. The most frequently used systemic medications are corticosteroids and cyclosporine, in monotherapy or in combination. Dapsone, azathioprine, cyclophosphamide, methotrexate, intravenous immunoglobulins, mycophenolate mofetil, and plasmapheresis are considered second-line agents. Hyperbaric oxygen, as supportive therapy, can be added. Anti-TNF-α agents such as etanercept, infliximab, and adalimumab are used in refractory cases. SS is usually responsive to oral corticosteroids, and the above-mentioned immunosuppressants should be considered in resistant or highly relapsing cases.

Clinical features and management of parastomal pyoderma gangrenosum in inflammatory bowel disease.

BACKGROUND: Pyoderma gangrenosum (PG) is often associated with inflammatory bowel disease even after bowel surgery, but it remains an extremely rare pathology. The purpose of this study was to investigate the clinical features and treatment of PG and to consider proper management for peristomal PG. METHODS: Demographic data for patients who underwent colorectal surgery with ostomy creation at Hyogo College of Medicine between July 2007 and July 2011 were prospectively collected. The main outcome measures were postoperative occurrence of peristomal PG by type: explosive and rapidly spreading type (type R) and indolent and gradually spreading type (type G). RESULTS: Overall prevalence was 11/738 (1.5%), with type R in 5 patients and type G in 6. Type R and type G were significantly more common in ulcerative colitis and Crohn's disease, respectively (p = 0.01). Type R developed within 6 days after surgery. Type G developed a mean of 52 days after surgery. Complete healing required a long time in both types, with means of 69 days for type R and 48 days for type G. CONCLUSION: Although peristomal PG was a rare complication after surgery, differences in the development of PG were observed between ulcerative colitis and Crohn's disease. Careful observation and knowledge of PG are needed.

Pyoderma gangrenosum: study of 21 patients and proposal of a 'clinicotherapeutic' classification.

BACKGROUND: Pyoderma gangrenosum (PG) is a rare, relapsing inflammatory disorder classified within the neutrophilic dermatoses. It can be idiopathic or associated with various conditions. The management of PG includes several immunosuppressants, but definite guidelines are still lacking. OBJECTIVE: To propose a 'clinicotherapeutic' classification for PG; namely, a therapeutic algorithm for this disease on the basis of the clinical extent of lesions. METHODS: Twenty-one patients with PG referred to our department during the last 3½ years were prospectively studied. They were subdivided into three subsets - localized, multilesional and disseminated - on the basis of the number of lesions and percentage of involved body surface area. RESULTS: The end point was fulfilled in all the aforementioned settings of PG. Topical tacrolimus proved to be useful in localized PG. Multilesional PG was successfully treated with prednisone alone or in combination with cyclosporine. Disseminated PG responded well to prednisone plus cyclosporine, except for refractory cases in which infliximab was employed. CONCLUSIONS: This clinicotherapeutic classification seems to work well in PG, although its impact on the incidence of relapses is poorly evaluable due to the short follow-ups in our study; controlled trials are needed to confirm its value.

Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is an idiopathic, ulcerative, noninfective chronic inflammatory skin disorder of unknown etiology. It is associated with systemic medical illness in 50% of cases like inflammatory bowel disease, systemic arthritis, haematological diseases and malignancies. Characteristic lesions begin as pustule or vesiculopustule and progresses to an ulcer or deep erosion with violaceous overhanging or undermined borders. Diagnosis of pyoderma gangrenosum is clinical and depends on exclusion of other causes of cutaneous ulceration. The management of PG is treatment of underlying systemic medical illness and judicious use of immunosuppressants. Association of PG with these medical illnesses and treatment with immunosuppressants make the clinical utility for internists, gastroenterologists, haematologists and rheumatologists.

[Superficial granulomatous pyoderma: report of a case of an uncommon variant of pyoderma gangrenosum]. / Pioderma granulomatoso superficial: relato de caso de variante rara do pioderma gangrenoso.

Pyoderma gangrenosum is a rare idiopathic skin disease. It affects mainly adults, and only 4% of the cases are diagnosed on children and adolescents. There are four clinical forms of pyoderma gangrenosum: ulcerative, pustular, bullous, and vegetative (superficial granulomatous pyoderma). Superficial granulomatous pyoderma is considered the most benign and uncommon form of the disease. Patients who have undergone surgical procedures may occasionally present pyoderma gangrenosum manifestations on the surgical site. A case of a five-year-old child, victim of burn, who presented superficial granulomatous pyoderma on the skin graft donor sites is reported.

Pioderma granulomatoso superficial: relato de caso de variante rara do pioderma gangrenoso / Superficial granulomatous pyoderma: report of a case of an uncommon variant of pyoderma gangrenosum

O pioderma gangrenoso é doença cutânea inflamatória rara, idiopática. Afeta principalmente adultos; apenas cerca de 4 por cento dos casos são diagnosticados em crianças e adolescentes. Existem quatro formas clínicas de pioderma gangrenoso: ulcerativa, pustular, bolhosa e vegetante (pioderma granulomatoso superficial). O pioderma granulomatoso superficial é considerado a forma mais benigna e incomum da doença. Em pacientes submetidos a manipulação cirúrgica, uma eventual manifestação do pioderma gangrenoso ocorre nos locais de intervenção. Relata-se o caso de criança de cinco anos de idade, vítima de queimadura, que apresentou pioderma granulomatoso superficial sobre áreas doadoras de enxertos.

Pyoderma gangrenosum is a rare idiopathic skin disease. It affects mainly adults, and only 4 percent of the cases are diagnosed on children and adolescents. There are four clinical forms of pyoderma gangrenosum: ulcerative, pustular, bullous, and vegetative (superficial granulomatous pyoderma). Superficial granulomatous pyoderma is considered the most benign and uncommon form of the disease. Patients who have undergone surgical procedures may occasionally present pyoderma gangrenosum manifestations on the surgical site. A case of a five-year-old child, victim of burn, who presented superficial granulomatous pyoderma on the skin graft donor sites is reported.

Pioderma gangrenoso: a propósito de 3 casos / Gangrenous pioderma: with regard to 3 cases

Se presentan 3 casos de pacientes con pioderma gangrenoso, 2 dos de ellos sin enfermedad sistémica asociada y 1 caso con diabetes concomitante. Se realiza una breve reseña de la clínica, histopatología, diagnóstico y tratamiento de esta patología.

Pyoderma gangrenosum--a review.

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. The treatment of PG is a challenge. Randomized, double-blinded prospective multicenter trials for PG are not available. The best documented treatments are systemic corticosteroids and cyclosporin A. Combinations of steroids with cytotoxic drugs are used in resistant cases. The combination of steroids with sulfa drugs or immunosuppressants has been used as steroid-sparing modalities. Anti-tumor necrosis alpha therapy in Crohn's disease showed a rapid response of PG. Skin transplants and the application of bioengineered skin is useful in selected cases as a complement to the immunosuppressive treatment. Topical therapy with modern wound dressings is useful to minimize pain and the risk of secondary infections. Despite recent advances in therapy, the prognosis of PG remains unpredictable.

From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research.

In 1964, Sweet described an acute febrile neutrophilic dermatosis. It is now widely accepted that Sweet's syndrome belongs to a group of associated neutrophilic dermatoses. Although clinically dissimilar, Sweet's syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, erythema elevatum diutinum, and a few other conditions can be considered a part of this same pathologic spectrum of inflammatory disorders because of (1) the existence of transitional and overlap forms; (2) the similar histopathologic feature of an infiltrate by normal polymorphonuclear leukocytes; (3) the possible occurrence of extracutaneous neutrophilic infiltrates, defining the neutrophilic disease; and (4) the frequent association with systemic diseases. According to the localization of the neutrophilic infiltrate, we describe neutrophilic dermatoses en plaques (dermal), superficial (epidermal), and deep (dermal and hypodermal). Almost every organ of the body may be involved by a neutrophilic aseptic inflammation. The main systemic diseases associated with neutrophlic dermatoses are hematologic, gastrointestinal, and rheumatologic diseases. Although the pathophysiology of these conditions is still poorly understood, treatment with systemic anti-inflammatory agents is usually efficacious.

Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a non-infectious reactive neutrophilic dermatosis which typically starts with pustules which rapidly evolve to painful ulcers of variable size and depth with undermined violaceous borders. Since its first description in 1930, the pathogenesis of PG has remained elusive even as an ever-widening range of systemic diseases has been described in association with it. The diagnosis of PG is based on clinical and pathologic features and requires exclusion of other conditions that produce ulcerations, since misdiagnosis exposes patients to risks associated with treatment. Critical to proper management are correct diagnosis, identification and treatment of any underlying disorder, and the appropriate choice of topical and systemic therapy. PG has four distinctive clinical and histologic variants, and the specific clinical features of the lesion may provide a clue to the associated disease. The most common associated diseases are inflammatory bowel disease, rheumatological or hematological disease or malignancy. Although there is no single successful treatment for PG, certain type of PG lesions are recognized to respond more readily to accepted therapies than others. Local treatment may be sufficient for mild disease, while systemic immunosuppressive therapy is necessary for severe cases. The treatments with the best clinical evidence are oral or pulse intravenous corticosteroids, and cyclosporine. Surgical therapy is useful in selected cases in conjunction with immunosuppression. Wound stabilization is obtained only through control of the systemic and local inflammatory process. Emerging therapies include use of platelet-derived growth factor and cell culture grafts when re-epithelialization is slow, and the TNF-alpha blocking agent infliximab for refractory disease. Despite advances in therapy, the long-term outcome for patients with PG remains unpredictable, because relapses are common.

[Cutaneous manifestations of internal diseases: purpura, livedo, pyoderma gangrenosum]. / Kutanmanifestationen internmedizinischer Erkrankungen: Purpura, Livedo, Pyoderma gangraenosum.

Dermatology and internal medicine have in common that many systemic diseases manifest with skin symptoms that are easily accessible for both diagnostic and investigative procedures. Even if modern practice of medicine tends towards organ specific particularity of specialists, dermatology traditionally strives for interdisciplinary communication. Cutaneous manifestations of internal diseases present in various forms. In a syndrome, a number of symptoms present in an identical pattern, and pattern recognition is diagnostic. Vice versa, a distinct skin sign can be a clue to various internal disorders, that have to be considered in the differential diagnosis. Purpura and livedo due to either inflammatory (immune complex mediated or associated with ANCA) or vasoocclusive microthrombotic vascular disease are presented as examples. Finally, subtile variations in key symptoms of the skin, such as of pyoderma gangrenosum, may give a clue to a specific underlying disease, such as rheumatoid arthritis, inflammatory bowel disease, monoclonal gammopathy, or hematologic disease, as exemplified by the variants of pyoderma gangrenosum.

Pioderma gangrenoso: a propósito de 6 casos / Pyoderma gangrenosum: report of 6 cases

Se presentan seis pacientes con pioderma gangrenoso ulceroso. Dos casos no tenían enfermedad sistémica asociada, el tercero presentaba un síndrome mielodisplásico y diabetes, el cuarto artritis reumatoidea y diabetes, el quinto síndrome mielodisplásico y hepatitis B y el sexto hepatitis C y síndrome hemofagocítico reactivo. Se describen las formas clínicas, enfermedades que pueden asociarse, histopatología, así como el diagnóstico y tratamiento de ésta infrecuente enfermedad (AU)

[Pyoderma gangrenosum. 15 cases]. / Pyoderma gangrenosum. A propos de 15 observations.

We report a retrospective study of 15 cases of pyoderma gangrenosum. The male/female ratio was 2, average age 40 years (range: 2-48 years). The typical ulcerous form was observed in 11 patients, whereas bullous pyodrma gangrenosum was noted in 2 patients and the granulomatous superficial form in 2 others. The leg was the most frequent localization. It was often associated with another skin localization. Two patients had neutrophilic pulmonary involvement which was concomitant to the skin ulcers. Association with internal disease was found in 8 patients. Histology showed vasculitis in 11 patients. In 9 of them, leukocytoclastic vasculitis was observed. Prednisone and clofazimine were the most frequently prescribed drugs. Th rate of recurrence of pyoderma gangrenosum was 46% independently of treatment. Occurrence of neutrophilic pneumopathy was rapidly fatal in two of our patients