Pyoderma Gangrenosum and Sterile Multifocal Osteomyelitis Preceding the Appearance of Takayasu Arteritis

Abstract: A 9-month-old infant had pyoderma gangrenosum (PG) and sterile osteomyelitis. Three years later the patient developed Takayasu arteritis (TA). Sterile osteomyelitis was reported in approxitnately 30 patients with different skin lesions, but never together with pyoderma gangrenosum. The association of PG and TA has been reported rarely, and then maiitly in adults or in children over age 9 years. This rare association should alert physicians with a case of infantile PG of unknown etiology to consider the possible diagnosis of TA.     

Infantile Crohn disease presenting with diarrhea and pyoderma gangrenosum

Cutaneous erosions and ulcerations in the diaper area are common in infancy and usually result from local irritation. We describe an infant with chronic diarrhea and failure to thrive who developed extensive ulcerations in the inguinal folds and perineum that were initially thought to be exclusively caused by local irritation. A cutaneous examination found signs consistent with those of pyoderma gangrenosum, leading to a diagnosis of infantile Crohn disease. Cutaneous signs can lead to the diagnosis of an underlying systemic disease in infants with chronic diarrhea and rash. Prompt diagnosis is especially important in infantile Crohn disease, since many infants require surgical resection of affected bowel, and 60% die from disease complications. This article reports a rare instance of an infant who developed pyoderma gangrenosum due to Crohn disease and reviews cutaneous signs of systemic disease in infants presenting with chronic diarrhea and rash.     

Idiopathic Infantile Pyoderma Gangrenosum with Stridor Responsive to Infliximab

Abstract:   A 4-month-old female infant presented with widespread pyoderma gangrenosum associated with stridor, presumed secondary to tracheal involvement. No underlying cause was revealed despite extensive investigation. Treatment with immunosuppressive agents only partially suppressed disease activity. Complete resolution followed treatment with infliximab. We review this rare condition in infants and discuss treatment with infliximab not previously described in this age group.     

Pyoderma gangrenosum in a patient with essential thrombocythemia

BACKGROUND: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum. OBJECTIVE: This article is a review of the associations between pyoderma gangrenosum and other diseases. RESULTS: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia. CONCLUSION: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.     

Strategies for optimizing treatment with efalizumab in moderate to severe psoriasis

BACKGROUND: Diagnosis of pyoderma gangrenosum can be difficult, leading to overdiagnosis or underdiagnosis. OBJECTIVE: To identify clinical features helpful in establishing a diagnosis of pyoderma gangrenosum and to compare the characteristics of patients with pyoderma gangrenosum with those of patients with chronic venous ulcers. METHOD: A retrospective chart review was performed in 28 patients with typical pyoderma gangrenosum and compared with the clinical features in 28 patients with chronic venous ulcers. RESULTS: (1) Even when other body sites are affected, pyoderma gangrenosum usually affects the upper and lower legs and feet or peristomal sites compared with chronic venous ulcers that are limited to the lower legs and feet. (2) Pyoderma gangrenosum can be associated with systemic diseases, especially inflammatory bowel disease. (3) Pustules and purulent discharge are features of pyoderma gangrenosum but not of chronic venous ulcers. (4) Crater-like holes or cribriform scarring is commonly seen in pyoderma gangrenosum but not in chronic venous ulcers. (5) Pathergy is a specific but not sensitive finding of pyoderma gangrenosum. It does not occur in patients with chronic venous ulcers. CONCLUSIONS: Diagnosis of pyoderma gangrenosum should be considered in patients with purulent ulcers affecting the legs or peristomal sites. To confirm the diagnosis, specific features should be sought, including pathergy, crater-like holes or cribriform scarring, and association with inflammatory bowel disease. Other causes of ulceration should be excluded.     

Pyoderma gangrenosum with pulmonary involvement?

Pyoderma gangrenosum is a rare, painful, noninfectious, ulcerative, reactive neutrophilic skin condition. It is characterized by ulcers that can spread quickly showing undermined violaceous borders. Since there is no single diagnostic test, early diagnosis is always challenging. The aggressive nature of classical pyoderma gangrenosum may become apparent only with time. Pulmonary involvement of pyoderma gangrenosum maybe underreported. We describe a case of classical pyoderma gangrenosum in a 65-year-old man with pulmonary involvement, who presented with a painful fluctuate nodule and ulcer with mucopurulent and hemorrhagic exudates, and with a monoclonal gammopathy, IgA type. One month later the ulcer and hemoptysis all disappeared after treatment with glucocorticosteroids. The pulmonary manifestations of pyoderma gangrenosum were also reviewed.     

Free flap coverage of pyoderma gangrenosum leg ulcers

Background: Pyoderma gangrenosum is a necrotizing skin disorder of unknown cause. Treatment of pyoderma gangrenosum usually entails medical treatments. Surgical treatment is generally not successful. Objective: Two patients with pyoderma gangrenosum of their lower extremities are presented. The application and utility of microvascular free flap coverage of their ulcers is discussed. Results: Both patients achieved successful healing with microvascular free flaps. Conclusion: Microvascular free flap coverage may be a surgical treatment option for selected patients with pyoderma gangrenosum.     

Lung involvement in pyoderma gangrenosum: a case report and review of the literature

Pyoderma gangrenosum is a neutrophilic dermatosis that, in addition to its characteristic skin manifestations, can cause visceral alterations. Our patient was a 34-year-old woman with pyoderma gangrenosum that was exacerbated by iodine and that also affected the lungs. Other published cases of lung involvement in pyoderma gangrenosum are reviewed and we discuss the possible exacerbation of this disease by iodine. Lung involvement is the most common extracutaneous manifestation of pyoderma gangrenosum and the main differential diagnosis is then with Wegener granulomatosis. It is important to remember the possible presentation of extracutaneous manifestations of pyoderma gangrenosum, including particularly lung involvement, in order to avoid subjecting a patient to aggressive diagnostic tests, at least in the initial stages.     

Successful treatment of pyoderma gangrenosum with topical 0.5% nicotine cream

Pyoderma gangrenosum is potentially a devastating and destructive disorder. There is no uniformly effective or specific therapy for pyoderma gangrenosum. Previous reports of nicotine therapy for pyoderma gangrenosum have suggested it to be efficacious. Unfortunately, previous reports were restricted by the use of commercially available preparations of nicotine, either as a gum or patch formulation. We have used topical nicotine 0.5% w/w cetamacrogol formula A cream that enables direct application onto the lesion, as well as dose and concentration variation. Two patients with pyoderma gangrenosum treated with topical nicotine 0.5% w/w cetamacrogol formula A cream are described here, both of whom had dramatic clinical resolution of their pyoderma gangrenosum.     

Ecthyma gangrenosum secondary to Staphylococcus aureus in an infant with transient neutropenia

Ecthyma gangrenosum is classically a cutaneous manifestation of a pseudomonal septicemia that presents in a patient with an immunodeficiency or hematologic malignancy. We describe a previously healthy 8-month-old girl who developed transient neutropenia and characteristic ecthyma gangrenosum lesions secondary to methicillin-resistant Staphylococcus aureus. This unique presentation of methicillin-resistant Staphylococcus aureus ecthyma gangrenosum emphasizes the importance of broad empiric coverage and early culturing for microorganism and susceptibilities in any patient presenting with ecthyma gangrenosum.     

Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia

Pyoderma gangrenosum is a neutrophilic dermatosis of unknown aetiology. Visceral involvement by pyoderma gangrenosum is rare, the lung being the most frequent site of extracutaneous disease. We describe a 73-year-old man with pyoderma gangrenosum and chronic myelomonocytic leukaemia in whom aseptic hepatosplenic abscesses and bony lesions were associated.     

Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non‐infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74‐year‐old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89‐year‐old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30–89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia‐localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia‐localized pyoderma gangrenosum tends to be without systemic complications.     

Pyoderma gangrenosum: a report of 15 cases and review of the literature

BackgroundPyoderma gangrenosum is a condition that is included among the neutrophilic dermatoses. Given its low incidence, few studies have addressed its epidemiology or treatment.ObjectiveTo describe the epidemiological and clinical characteristics of patients with pyoderma gangrenosum along with our experience of treating the condition in a referral hospital in Malaga, Spain.Material and methodsA retrospective, observational study was undertaken in the Department of Dermatology at Hospital Clínico Universitario Virgen de la Victoria in Malaga, Spain between January 2000 and December 2009 and included all patients diagnosed with pyoderma gangrenosum.ResultsThe incidence of pyoderma gangrenosum in our reference population is 3.26 cases per million inhabitants per year. The most frequent concomitant systemic disease was ulcerative colitis (5 cases, 33%). In 4 patients with that disease, pyoderma gangrenosum appeared during a flare-up. In 80% of cases, patients were not referred to a dermatologist during the initial phase of pyoderma gangrenosum, and most referrals were from gastroenterology or general surgery (4 patients each, 52%).ConclusionsPatients with pyoderma gangrenosum are often referred to dermatologists by other specialists after a varying period of time has elapsed without achieving an accurate diagnosis. In these patients, especially those between 20 and 40 years of age, it is essential to rule out concomitant disease. Adalimumab is a good treatment option for pyoderma gangrenosum.     

Familial ulcerative pyoderma gangrenosum: a report of 2 kindred

Pyoderma gangrenosum is a rare, chronic ulcerative skin disease. It is a diagnosis of exclusion, after ruling out other causes of cutaneous ulceration. The etiology of pyoderma gangrenosum is poorly understood but is likely multifactorial. We describe 2 families affected by ulcerative pyoderma gangrenosum. This familial clustering suggests a possible genetic role in the development of pyoderma gangrenosum in some cases.     

Pyoderma gangrenosum in an infant: A case report and review of the literature

Pyoderma gangrenosum is a neutrophilic dermatosis that is rare in infancy, with only 20 cases reported in the literature. We present a case of infantile pyoderma gangrenosum refractory to topical steroids, tacrolimus, and dapsone as well as systemic steroids and infliximab that is currently well controlled with the addition of oral tacrolimus. To our knowledge, this is the first report of the effective, safe use of oral tacrolimus in combination with infliximab for infantile pyoderma gangrenosum. We review all current cases of infantile pyoderma gangrenosum, as well as tacrolimus and its role in the treatment of this condition.     

Multiple cavitary pulmonary nodules in association with pyoderma gangrenosum: case report

Pyoderma gangrenosum is a rare neutrophilic disease of unknown origin that is associated with systemic diseases in 50% of cases. It is characterized by erythematous-violaceous nodular lesions that quickly progress to painful ulcers, with undermined edges, necrotic-hemorrhagic, varying in size and depth, located mainly in the lower limbs. Extracutaneous locations of pyoderma gangrenosum are rare, usually involving the lungs; the main differential diagnosis in these cases is Wegener granulomatosis. We report a case of pyoderma gangrenosum, which showed multiple cavitary lung nodules, with good response to high doses of steroids. Once excluded the possibility of Wegener granulomatosis, the authors concluded that it was the manifestation of systemic pyoderma gangrenosum with pulmonary involvement.     

Pyoderma gangrenosum or cutaneous lymphoma: a difficult clinical diagnosis

Pyoderma gangrenosum is frequently associated with an underlying condition such as ulcerative colitis or a myeloproliferative syndrome. However, lymphoproliferative malignancies have only rarely been seen concurrently with pyoderma gangrenosum. We present the case of a patient with small lymphocytic lymphoma who noted a slowly enlarging skin ulcer that was clinically consistent with pyoderma gangrenosum. Examination of a biopsy specimen showed infiltration of the skin with lymphoma cells as well as deeper necrotic material and thrombosis of vessels that were infiltrated by lymphoma. This case illustrates the difficulty of differentiating pyoderma gangrenosum from cutaneous lymphoma clinically.     

Ecthyma gangrenosum without bacteraemia in a leukaemic patient

Ecthyma gangrenosum is a well recognized cutaneous manifestation of Pseudomonas aeruginosa infections in immunocompromised patients. Most cases of ecthyma gangrenosum have been associated with concomitant septicaemia. However, ecthyma gangrenosum rarely develops due to Ps. aeruginosa in the absence of bacteraemia. We report a rare case of a nonsepticaemic form of ecthyma gangrenosum presenting as a large solitary necrotic ulcer in a patient with acute myelogenous leukaemia. A culture from the lesion revealed the presence of Ps. aeruginosa, but the results of repeated blood cultures were negative. Histological examination revealed numerous tiny eosinophilic bacilli in the dermis and panniculus with Gram's stain.     

Pustular pyoderma gangrenosum: an uncommon variant which is easily misdiagnosed

Pustular pyoderma gangrenosum is a relatively uncommon clinical form of pyoderma gangrenosum; it presents with vesiculo-pustular lesions that do not develop into frank ulceration. We report a case of a 44-year-old man with associated ulcerative colitis, who was misdiagnosed as having necrotizing fasciitis. He underwent multiple debridements and a subsequent skin grafting procedure, but without improvement. The diagnosis of pyoderma gangrenosum is often challenging because there is no defining diagnostic clinical, laboratory, or histopathological feature. A high index of suspicion is, therefore, essential to diagnose pyoderma gangrenosum clinically because failure to do so in the early stages of the disease can lead to disfigurement and even unnecessary and detrimental surgery.     

Present status of pyoderma gangrenosum. Review of 21 cases

This article summarizes the management of 22 cases of pyoderma gangrenosum over the past four years at the hospital of the University of Pennsylvania, Philadelphia. Eighteen patients with pyoderma gangrenosum were studied using the most sensitive routine laboratory method for detection of monoclonal immunoglobulins, immunofixation electrophoresis. Four cases of IgA gammopathy were detected, confirming previous reports of the incidence of monoclonal gammopathy in pyoderma gangrenosum. High-dose glucocorticoid therapy (pulse therapy) is an effective treatment for some severe, refractory cases of pyoderma gangrenosum. Eight patients were treated with pulse therapy. Six responded favorably, and none had serious complications.