Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Pyoderma Gangrenosum”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,261 records · Page 70Linked to original sources
PubMed1995

Paraneoplastic autoimmune phenomena in patients with myelodysplastic syndromes: response to immunosuppressive therapy.

We analysed the clinical features, course and response to immunosuppressive therapy in 30 patients with autoimmune disorders associated with myelodysplastic syndromes (MDS). 18 patients with MDS developed acute systemic autoimmune disorders. Common manifestations were skin vasculitis (n = 15) and arthritis (n = 11). Seven patients had an acute clinical syndrome of vasculitic skin rash, fever and arthritis with peripheral oedema in three and pulmonary infiltrates in five of these seven patients. Other acute manifestations included pericarditis, pleural effusions, skin ulceration, seizures, myositis and peripheral neuropathy. Chronic or isolated autoimmune manifestations (n = 11) included glomerulonephritis, polyneuropathy, pyoderma gangrenosum, ulcerative colitis and polyarthritis. Classic connective tissue disorders recognized included relapsing polychondritis, polymyalgia rheumatica, Raynaud's syndrome and Sjögren's syndrome. Autoimmune manifestations responded to immunosuppressive therapy (primarily prednisone) in 26/27 patients treated. Furthermore, cytopenias improved substantially in six patients, including complete normalization of peripheral blood counts in two patients with cytogenetic remission in one. Patients with a haematological response to immunosuppressive therapy had improved survival compared with non-responding patients. The autoimmune syndrome was implicated as a primary cause of death in 8/17 patients who died. Autoimmune manifestations may be more common than previously recognized in patients with MDS. Aggressive therapy with immunosuppressive agents in selected patients often controls autoimmune phenomena associated with MDS and may lead to haematological responses in some patients.

Adolescent↗
PubMed1996

Metastatic Crohn's disease of the umbilicus.

We report a case of metastatic Crohn's disease of the umbilicus which responded to topical corticosteroid treatment. Crohn's disease is a granulomatous disease of the bowel which may affect other organs. The skin is commonly involved, and the cutaneous manifestations may be non-specific, e.g. pyoderma gangrenosum or erythema nodosum, or specific with epithelioid granulomas and multinucleated giant cells in the skin. Specific skin lesions may be contiguous with the bowel at sites including the perineum, mouth and adjacent to fistulae, or separated from it by normal skin from the bowel, a rare condition which has been termed 'metastatic' Crohn's disease.

Aged↗
PubMed1995

Histologic spectrum of cutaneous involvement in patients with myelogenous leukemia including the neutrophilic dermatoses.

BACKGROUND: Cutaneous manifestations of myeloid leukemia can be specific or nonspecific. The study was designed to determine the prevalence and histologic appearance of cutaneous lesions in patients with myeloid leukemia and various myeloproliferative disorders. METHODS: The histologic changes of cutaneous lesions in 52 patients with myelodysplastic syndrome, polycythemia vera, and myeloid, myelomonocytic, or monocytic leukemia are presented in this study. RESULTS: Two types of cellular infiltrates were identified. In the first group, the most common pattern was a diffuse involvement by the leukemic cells through the entire dermis with preservation of a "grenz zone" in the superficial dermis. Two cases exhibited a Kaposi's sarcoma-like pattern, with prominent slit-like blood-filled spaces lined by myeloblasts against a fibrocellular stroma. The second group of lesions was characterized by dense, neutrophilic dermal infiltrates resembling acute neutrophilic dermatosis (Sweet's syndrome) or pyoderma gangrenosum. In two of these cases, scattered immature blast cells admixed with the mature neutrophilic elements were identified. CONCLUSIONS: Awareness of these different morphologic features and application of special stains are of value in the evaluation of suspicious cutaneous infiltrates in patients with myeloid leukemia and various myeloproliferative disorders.

Adult↗
PubMed2004

Granulocyte adsorptive apheresis for leg ulcers complicated by rheumatoid arthritis: a report on three successfully treated cases.

BACKGROUND: The treatment of inflammatory leg ulcers complicated by rheumatoid arthritis (RA), which are unresponsive to conventional care, can be frustrating. Furthermore, as granulocytes and monocytes (GM) are major sources of inflammatory cytokines, they have the potential to initiate and perpetuate inflammatory skin lesions. Accordingly, a recent study reported the remission of pyoderma gangrenosum following the reduction of activated peripheral blood GM by adsorptive apheresis (GMA). METHODS: In this clinical study, we applied GMA to three cases, each with one leg ulcer below the knee and RA. The ulcers had not responded to conventional therapy, including disinfection, dressing, and antimicrobials, and therefore were thought to represent inflammatory vasculitic lesions. GMA was performed using a column with a capacity of 335 mL, filled with cellulose acetate beads that selectively adsorb granulocytes and monocytes/macrophages (Adacolumn). Each patient received one GMA session/week for five consecutive weeks. The duration of one session was 60 min, with a flow rate of 30 mL/min. RESULTS: The ulcers began to recede after two GMA sessions and, by the end of the fifth session, the ulcers in all three patients had healed. No recurrence has been observed up to the time of this report. The treatment was well tolerated and no severe side-effects were observed. CONCLUSIONS: GMA, which depletes activated neutrophils and monocytes/macrophages, appears to be effective for inflammatory skin ulcers which do not respond to conventional medications.

Aged↗
PubMed2004

Cutaneous sarcoidosis and polycythemia vera.

Polycythemia vera is classified with myelogenous leukaemia, agnogenic myeloid metaplasia and primary thrombocythemia as a myeloproliferative syndrome. Cutaneous symptoms have been reported with polycythemia vera, including facial plethora, aquagenic pruritus, urticaria, purpura, Sweet's syndrome and pyoderma gangrenosum. However, polycythemia vera associated with systemic sarcoidosis has been rarely reported. An unusual case of polycythemia vera associated with cutaneous sarcoidosis is described.

Aged↗
PubMed2004

Smoking can be good for you.

Smoking is without doubt one of the greatest causes of avoidable illness and death in the modern world. Most well known is the relationship between smoking and numerous cancers, cerebrovascular and cardiovascular disease. Smoking and most especially nicotine, are, however, sometimes beneficial in certain diseases, including Parkinson's, Alzheimer's, allergic alveolitis, nausea and vomiting of pregnancy, pre-eclampsia, fibroids, carcinoma of body of uterus, ulcerative colitis, pyoderma gangrenosum, aphthous stomatitis and ulceration, pemphigus, herpes simplex and acne. In the immensely justifiable enthusiasm to discredit this dangerous activity, the mechanisms behind these beneficial effects tend to have been un-discussed or ignored. It is the aim of this paper to spur interest in the reasons for these effects. If the mechanisms are elucidated, therapeutic advances may be possible.

Journal Article↗
PubMed1993

Inflammatory ulcers.

A large number of diseases can eventuate in cutaneous ulceration. This article will review inflammatory disorders which by their nature can directly produce cutaneous breakdown and ulcer formation. Major emphasis is given to those disorders where recent knowledge has improved our understanding of the condition or where new therapeutic agents or maneuvers have become available. This later group consists of vasculitis, disorders caused by small vessel thrombi or embolus and pyoderma gangrenosum.

Humans↗
PubMed1992

Cutaneous manifestations of pediatric HIV infection.

Children who are infected with human immunodeficiency virus may develop a wide variety of mucocutaneous manifestations, such as skin infections, tumors, and inflammatory skin disorders. The most significant infectious diseases are candidiasis, dermatophytosis, herpes simplex, herpes zoster, and pyoderma. Inflammatory disorders include seborrheic dermatitis, vasculitis, and pyoderma gangrenosum. Kaposi sarcoma is extremely rare in children with the acquired immunodeficiency syndrome.

AIDS-Related Opportunistic Infections↗
PubMedDate not supplied

Basic approach to inflammatory ulcers.

Cutaneous ulcers are a difficult problem. A multidisciplinary approach is often adapted in caring for patients with nonhealing wounds. Internists, surgeons, dermatologists, and nurses must work together to provide a patient with the most comprehensive care possible. Dermatologists are consulted for a number of reasons, particularly if the ulcers are secondary to an inflammatory process. In this discussion, we describe a basic and useful approach to the diagnosis and management of inflammatory ulcers. The spectrum of these conditions is wide, ranging from vasculitis, to pyoderma gangrenosum, to ulcers resulting from the antiphospholipid syndrome and cryoprecipitates. The present authors will emphasize that the approach needs to be all-encompassing, and not focused on the ulceration alone. Certain clues are critical to diagnosis. The management, whereas often difficult, can be very rewarding.

Humans↗
PubMed1998

Histopathology of cutaneous reaction to granulocyte colony-stimulating factor: another pseudomalignancy.

Granulocyte colony-stimulating factor (G-CSF) is a hematopoietic growth factor (HGF) with many applications in cancer therapy. The most important applications are reduction in the incidence of febrile neutropenia, acceleration of neutrophil recovery after chemotherapy or bone marrow transplantation, and mobilization of progenitor cells. Many cutaneous adverse reactions associated with HGF have been reported in recent years, including injection site reactions, pyoderma gangrenosum, Sweet's syndrome, cutaneous leucocytoclastic vasculitis, and widespread folliculitis. The presence of large histiocytes on the dermis between collagen bundles has been proposed as a characteristic histopathologic finding in cutaneous eruptions secondary to granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor. We report on a patient with a high-risk ductal infiltrating carcinoma of the breast who received high-dose chemotherapy (HDC) with peripheral blood progenitor cell (PBPC) rescue. The patient received G-CSF after PBPC for a faster granulocyte recovery. She developed a cutaneous eruption located on back, buttocks, axillae, groin and sites where electrocardiography electrodes had been placed. From the histopathological point of view, the eruption was characterized by the presence of numerous large, atypical histiocytes in the dermis with several mitotic figures, mimicking involvement of the dermis by a malignant process.

Breast Neoplasms↗
PubMed2006

Expression of cathepsin L and its inhibitor hurpin in inflammatory and neoplastic skin diseases.

Cathepsin (Cat) L is an important lysosomal proteinase involved in a variety of cellular functions including intracellular protein turnover, epidermal homeostasis and hair development. Hurpin (serpinB13) is a cross-class specific serine protease inhibitor of Cat L. We have analysed the expression and localization of Cat L and hurpin in various inflammatory and neoplastic diseases by immunohistochemistry. Whereas Cat L expression in normal skin was below detection limit, immunoreactivity was detected in chronic inflammatory dermatoses. The highest expression of Cat L was found in psoriasis, atopic eczema and squamous cell carcinoma (SCC) samples. Samples of Lupus erythematosus, folliculitis, acne inversa, chronic leg ulcers and pyoderma gangrenosum demonstrated similar but lower expression for Cat L. In malignant cells of SCC, basal cell carcinoma and malignant melanoma, characteristic staining patterns were observed for Cat L, with more abundant expression at the periphery of the tumor. Expanding our previous work, we found that the expression of hurpin was confined mainly to the basal layer in normal skin samples, whereas hurpin was overexpressed and redistributed in diseased skin. The localization of hurpin in dermatoses and neoplasias differed from that in normal skin in that the highest expression was found in the outermost layers of the granular and upper spinous layers. Similarly to Cat L, the highest expression for hurpin was found in psoriasis and SCC. The results presented here summarize for the first time the expression of the protease Cat L and its inhibitor hurpin in a broad spectrum of skin diseases.

Carcinoma, Squamous Cell↗
PubMed2005

Segmental ulcerative vasculitis: a cutaneous manifestation of Takayasu's arteritis.

A 16-year-old girl with pyoderma gangrenosum (PG)-like skin lesions on the extremities, trunk and face developed Takayasu's arteritis (TA; pulseless disease). After 3 years under maintenance cyclosporin A therapy, the patient developed an ischaemic cerebral accident. Severe obstruction of both subclavian and left carotid arteries was found by Doppler sonography, angiography and computerised axial tomography. Evolution of this disease showed some characteristic findings: (a) PG-like lesions as the first cutaneous manifestation of pulseless disease; (b) methotrexate and cyclosporin A giving good results for the cutaneous lesions, but apparently not exerting an influence on the evolution of TA and the fatal outcome. This morphologic pattern may reflect underlying TA or Wegener's arteritis, and should be termed segmental ulcerative vasculitis.

Adolescent↗
PubMed2007

Autoinflammatory gene mutations in Behçet's disease.

BACKGROUND: Behçet's disease (BD) shares clinical features with well-recognised autoinflammatory disorders. In addition, mutations in genes for familial Mediterranean fever and tumour necrosis factor receptor-associated periodic syndrome have been reported to have increased in patients with BD. PATIENTS AND METHODS: DNA samples from 97 patients with BD and 51 matched healthy controls were analysed for the mevalonate kinase (MVK), cold-induced autoinflammatory syndrome 1 (CIAS1) and proline/serine/threonine phosphatase-interacting protein 1 (PSTPIP1) genes, responsible for mevalonate kinase deficiency (MKD), cryopyrin associated periodic syndromes (CAPS) and pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) syndrome, respectively. Over 90% of known mutations were screened using restriction fragment length polymorphism analysis and/or sequencing. RESULTS: Two patients had paired mutations in the MVK gene (genotypes V377I/V377I and V377I/S135L) and displayed typical features of BD and MKD. Another was heterozygotic for the V377I genotype. The V198M mutation in the CIAS1 gene was identified in one patient with typical BD but no symptoms of CAPS. No mutations were identified in the control group. PSTPIP1 analysis revealed a new exon 10 insertion variant (c.741+33_741+34insGT) in 2 of 97 patients and in 1 of 51 controls (p>0.05), indicating that it is a polymorphism rather than a true mutation. DISCUSSION: This study could not demonstrate any significant increases in MVK, CIAS1 or PSTPIP1 mutations in patients with BD as compared with controls.

Adaptor Proteins, Signal Transducing↗
PubMed2006

Pathogenesis of familial periodic fever syndromes or hereditary autoinflammatory syndromes.

Familial periodic fever syndromes, otherwise known as hereditary autoinflammatory syndromes, are inherited disorders characterized by recurrent episodes of fever and inflammation. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to very minor stimuli or turned off too late. The genetic background of the major familial periodic fever syndromes has been unraveled, and through research into the pathophysiology, a clearer picture of the innate immune system is emerging. After an introduction on fever, interleukin-1beta and inflammasomes, which are involved in the majority of these diseases, this manuscript offers a detailed review of the pathophysiology of the cryopyrin-associated periodic syndromes, familial Mediterranean fever, the syndrome of pyogenic arthritis, pyoderma gangrenosum and acne, Blau syndrome, TNF-receptor-associated periodic syndrome and hyper-IgD and periodic fever syndrome. Despite recent major advances, there are still many questions to be answered regarding the pathogenesis of these disorders.

Animals↗
PubMed1998

Abscess-forming neutrophilic dermatosis: report of three cases associated with hemopathies.

BACKGROUND: The development of different types of neutrophilic dermatosis is reported to occur in the course of malignant hemopathies. These concern mainly Sweet's syndrome, pyoderma gangrenosum, erythema elevatum et diutinum and neutrophilic eccrine hidradenitis. OBSERVATIONS: We have recently encountered the cases of 3 patients who presented all with multiple acneiform papules and dome-shaped aseptic abscesses leaving scars. Pus was sterile in all except case 3 in which slight Staphylococcus aureus growth was shown. However, in this patient, only steroids were effective demonstrating that this bacterium was not responsible for the disease. Histopathology disclosed a dense dermal polymorphonuclear neutrophil infiltrate and some mononuclear cells. Two of these patients had myelodysplastic syndromes while one had IgA myeloma. CONCLUSION: Abscess-forming neutrophilic dermatosis seems to be another type of neutrophilic dermatosis associated with hematological malignancies.

Abscess↗
PubMed2000

Leg ulcers in patients with myeloproliferative disorders: disease- or treatment-related?

Leg ulcers are a relatively frequent problem in patients with myeloproliferative disorders under treatment with hydroxyurea (HU). The pathogenesis is currently unknown and may be multifactorial. Concomitant arterial or venous disease may play a contributing role in the development of these wounds. Vasculitis, cryoglobulinemia and pyoderma gangrenosum should be considered if typical clinical signs are present. We report on 3 patients with myeloproliferative disorders who developed HU-induced leg ulcers and review the literature. HU-induced leg ulcers share clinical features which can help to differentiate them from leg ulcers of other etiologies: occurrence under long-term treatment with HU at a dose of at least 1 g/day, localization in the malleolar region and spontaneous healing when HU is discontinued. We conclude that differentiation between disease-related and treatment-induced leg ulcers can be difficult and may not always be possible. In HU-induced leg ulcers, cessation of the drug typically leads to wound healing.

Aged↗
PubMed2000

Takayasu arteritis and cutaneous necrotizing vasculitis.

Takayasu arteritis (TA) is an inflammatory arteriopathy involving predominantly the aorta and its main branches. The disease evolves in two phases: a first, nonspecific inflammatory stage and a late 'pulseless' stage, in which complications related to arterial stenosis and aneurysm formation predominate. In both phases, skin manifestations, such as inflammatory nodules, erythema-nodosum- and pyoderma-gangrenosum-like ulcers, have been described. We report 2 patients with TA, who had cutaneous necrotizing vasculitis as presenting manifestation of the disease. A review of the literature revealed 8 similar cases. TA does not only involve large arteries, but also small blood vessels. The observation that in TA the inflammatory process of the large arteries affects regions of the walls supplied by the vasa vasorum, the anatomy of which bears resemblance to the cutaneous vessel system, suggests that primary involvement of small vessels contributes to the development of the clinicopathological features of TA. Knowledge of the skin manifestations associated with TA remains important for its diagnosis and prompt instauration of life-saving treatment.

Adult↗
PubMed2004

Takayasu's disease with cutaneous involvement.

Takayasu's arteritis (TA) is a chronic inflammatory and fibrosing arteriopathy that can also involve cutaneous vessels. The disease typically presents with a prepulseless phase that overlaps or is followed by the characteristic pulseless stage. In both phases of TA, cutaneous manifestations may be present. Lesions considered to be 'specifically' associated with TA have been described most frequently simulating erythema nodosum, erythema induratum and pyoderma gangrenosum. We report 2 Caucasian patients with TA and nodular cutaneous lesions. Nine skin biopsies from these patients were studied. A necrotizing vasculitis was present in 5 biopsies. We review those patients with TA and well-documented cutaneous manifestations in the English literature, with special interest in nodular lesions, the most frequent cutaneous manifestation of TA in Caucasian patients. Biopsies from lesions with similar morphology frequently show different histological findings.

Adult↗