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 1,189 records · Page 66Linked to original sources

Diffuse febrile dermatosis in a patient with active ulcerative colitis under treatment with steroids and azathioprine: a case of Sweet's syndrome. Case report and review of literature.

Ulcerative colitis is an inflammatory bowel disease often associated with extra-intestinal manifestations, such as dermatological disorders. Of these, the most frequent are erythema nodosum and pyoderma gangrenosum, the two neutrophilic forms of dermatosis. Another is Sweet' s syndrome, which results in a sudden eruption of tender, raised erythematous or violaceous plaques/papules or nodules, less frequent vesicles, pustules or bullae, involving face, neck, arms and trunk. This skin disorder is frequently observed in patients with leukaemia or connective tissue diseases, while it is very rare in patients with inflammatory bowel disease. The present report deals with the case of a febrile diffuse skin eruption in a 53-year-old patient with moderately active ulcerative colitis after few days' treatment with steroids and azathioprine. At first, the dermatosis was addressed to an idiosyncrasy to azathioprine, which was, therefore, promptly discontinued. Histological examination of skin biopsies revealed the presence of features typical of a Sweet's syndrome. The eruption gradually improved as well as the patient's general condition, until complete regression was achieved following steroid treatment.

Azathioprine↗

Iontophoresis of lecithin vesicles of cyclosporin A.

Site-specific immunosuppression with topical cyclosporin A (CsA) has broad clinical implications in the treatment of skin disorders like psoriasis, pyoderma gangrenosum, lichen planus, cutaneous graft-versus-host disease and contact hypersensitivity and the temporary treatment of skin allografts on burn wounds. However, like any other peptide drug, its skin delivery is hindered by the barrier property of stratum corneum and the physicochemical properties of CsA. We have attempted to deliver CsA across human cadaver epidermis in vitro using colloidal systems like microemulsion and lecithin vesicles and iontophoresis. Although, passive diffusion did not result in permeation of quantifiable amounts of CsA, anodal iontophoresis of the negatively charged colloidal systems facilitated the permeation. Electroosmosis and compromised epidermis might have contributed to the higher skin flux. Lecithin vesicles were better than microemulsion for the iontophoretic delivery of CsA and appear to have potential in site-specific immunosuppression.

Cyclosporine↗

A common pathway in periodic fever syndromes.

Familial Mediterranean fever (FMF) is an autosomal recessive disease due to mutations in pyrin, which normally inhibits pro-interleukin-1beta (IL-1beta) cytokine processing to the active form. A novel role for pyrin has been proposed by Shoham et al., who studied patients with an autosomal dominant disease called pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. They demonstrated an interaction between pyrin and proline serine threonine phosphatase-interacting protein 1 (PSTPIP1), the protein involved in PAPA, and thus revealed a biochemical pathway common to both FMF and PAPA.

Adaptor Proteins, Signal Transducing↗

Nonhealing ulcer secondary to factor V Leiden mutation and cryofibrinogenemia.

Factor V Leiden is the most common genetic thrombophilia in people of European descent, and is important to recognize as it can have significant implications in dermatology. We report a case of a 30-year-old man who presented for evaluation and treatment of a chronic ulceration on the site of his stump following a below the knee amputation which had been performed for non-healing ulcerations. Despite a variety of treatments, his ulcer persisted. He was referred to a dermatologist who performed a biopsy that was interpreted ass non-specific, and treatment was started for pyoderma gangrenosum. Further investigation revealed a homozygous factor V Leiden mutation and cryofibrinogenemia. He was tapered off of the methylprednisolone and was improving on stanozolol. He healed well after surgery and no new ulcerations have developed. This case highlights the importance of considering this mutation in a non-healing leg ulcer.

Adult↗

Rheumatoid arthritis: a review of the cutaneous manifestations.

Rheumatoid arthritis is a chronic inflammatory arthritis with significant extra-articular manifestations. Of note are unique cutaneous manifestations that the dermatologist may encounter. This article will make the dermatologist more cognizant of these skin findings in patients with this systemic inflammatory disorder. It examines rheumatoid arthritis, focusing on the general nonspecific and disease-specific rheumatoid arthritic skin changes. Classic rheumatoid nodules, accelerated rheumatoid nodulosis, rheumatoid nodulosis, rheumatoid vasculitis, Felty syndrome, pyoderma gangrenosum, interstitial granulomatosus dermatitis with arthritis, palisaded neutrophilic and granulomatosis dermatitis, rheumatoid neutrophilic dermatitis, juvenile rheumatoid arthritis, and adult-onset Still disease are reviewed. Understanding the cutaneous expressions of rheumatoid arthritis may lead to early diagnosis, prompt treatment, and lower morbidity and mortality for the affected persons. Learning objective At the completion of this learning activity, participants should be able to describe rheumatoid arthritis in terms of its epidemiology, etiology, pathogenesis, and general and specific cutaneous manifestations.

Arthritis, Rheumatoid↗

Tetracyclines: nonantibiotic properties and their clinical implications.

Tetracyclines are broad-spectrum antibiotics that act as such at the ribosomal level where they interfere with protein synthesis. They were first widely prescribed by dermatologists in the early 1950s when it was discovered that they were effective as a treatment for acne. More recently, biologic actions affecting inflammation, proteolysis, angiogenesis, apoptosis, metal chelation, ionophoresis, and bone metabolism have been researched. The therapeutic effects of tetracycline and its analogues in various diseases have also been investigated. These include rosacea, bullous dermatoses, neutrophilic diseases, pyoderma gangrenosum, sarcoidosis, aortic aneurysms, cancer metastasis, periodontitis, and autoimmune disorders such as rheumatoid arthritis and scleroderma. We review the nonantibiotic properties of tetracycline and its analogues and their potential for clinical application.

Acne Vulgaris↗

The 5q- syndrome and autoimmune phenomena: report of three cases.

Myelodysplastic syndrome is a clonal hematopoietic stem cell disorder characterized by ineffective hematopoiesis, peripheral cytopenias and an additional risk to evolve to acute leukemia in up to 30% of the cases. Autoimmune manifestations as vasculitis, pyoderma gangrenosum, hemolytic anemia, immune thrombocytopenia, rheumatoid arthritis as well as positive anti-nuclear factor and rheumatoid factor have been reported in 13-30% of MDS patients. The aim of this report is to present three patients with 5q- syndrome who presented different autoimmune serological and clinical phenomena and review the literature. Patient 1 showed a focal and segmental glomerulosclerosis (FSGE) in the course of a MDS. Renal involvement in MDS as autoimmune phenomenon is rare and few reports have documented different forms of glomerular diseases in adults with MDS. Patients 2 and 3 showed a rheumatoid factor of 1/140 and the direct Coomb's test positive (3+), respectively, but without evidence of clinical autoimmune manifestation. In conclusion, patients with the 5q- syndrome experience a relative benign disease course extending over several years. We believe that careful follow-up of patients with autoimmune manifestations as here reported is important to detect any unexpected outcome.

Aged↗

[Aortitis after G-CSF injections].

INTRODUCTION: Aortitis consists in aortic wall inflammation from infectious or non infectious cause. It may lead to aortic aneurysm with a risk of rupture, which is life-threatening and may justify surgical procedures. The cause of the aortitis is sometimes obscure. CASE REPORT: We report the case of a 55 years old woman who developed acute aortitis of the descending aorta after G-CSF (granulocyte-colony stimulating factor) injections for blood stem cells graft. No cause was found to the aortitis, the evolution was favorable after corticosteroid treatment, without aneurysm at six months. CONCLUSION: The present case rises the question of G-CSF (Neupogen responsibility in aortic lesions. Neutrophilic mediated diseases (Sweet's syndrome, pyoderma gangrenosum) and leukocytoclastic vasculitis were reported after G-CSF therapy. Neutrophils induced by G-CSF injections present functional abnormalities which may play a role in the pathogenesis of these diseases.

Aortitis↗

[Neutrophilic dermatoses].

PURPOSE: The neutrophilic dermatoses are a group of skin diseases mainly characterized by an infiltration of the skin by normal polymorphonuclears, without any identifiable (infectious) cause. In this review we describe the main neutrophilic dermatoses and point on their multisystemic dimension. CURRENT KNOWLEDGE AND KEY-POINTS: Well-defined neutrophilic dermatoses are Sweet's syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, erythema elevatum diutinum and neutrophilic eccrine hidradenitis, as well as newer and rarer conditions such as aseptic abscesses. Clinical overlap between these disorders led to the concept of "the" neutrophilic dermatosis. In addition, patients with a neutrophilic skin disorder may also suffer from extra-cutaneous aseptic neutrophilic infiltrates. This "neutrophilic disease" is often difficult to diagnose. Neutrophilic dermatoses are significantly associated with systemic disease. These include hematological disorders, inflammatory bowel disease, joint immunological disorders, and other malignant or inflammatory conditions. First-line treatment of acute neutrophilic conditions is steroid therapy. In chronic conditions, dapsone is often efficient. FUTURE PROSPECTS AND PROJECTS: The mechanisms underlying the inappropriate activation of polymorphonuclears are poorly understood. Hematopoietic growth factors and adhesion molecules are believed to play a role in the pathophysiology of the neutrophilic dermatoses.

Abscess↗

Biologic-biologic and biologic-JAK inhibitor combination therapy in refractory systemic autoinflammatory diseases.

OBJECTIVES: Systemic autoinflammatory diseases (SAIDs) arise from genetic defects in innate immunity, leading to dysregulated activation of inflammatory pathways, including interleukin (IL)-1, IL-6, TNF, and JAK/STAT. Clinical manifestations range from recurrent fever to severe complications such as encephalitis and AA amyloidosis. Management aims to control inflammation using immunosuppressive agents and targeted monotherapies (biologics or JAK inhibitors). Advanced combination therapy (ACT), defined as the use of biologics and/or JAK inhibitors in combination, has emerged as a strategy for refractory disease. METHODS: In this observational retrospective longitudinal cohort study, patients with SAIDs treated with ACT were included. Demographic, clinical, treatment, and safety data were collected. Treatment response was assessed using a composite outcome incorporating corticosteroid dose, C-reactive protein (CRP), and clinical improvement and categorized as non-response, partial response, or complete response. RESULTS: Thirty-eight patients (median age 30 years [range 4-76]) were included. The most common indications for ACT were pyogenic arthritis, pyoderma gangrenosum and acne (PAPA), mevalonate kinase deficiency (MKD), and undifferentiated SAIDs. Most patients had disease-related complications and were dependent on glucocorticoids and/or opioids to control inflammation and pain, respectively. Following multiple ACT trials, complete response was observed in 21 patients (55.3%), partial response in 12 (31.6%), and no response in 5 (13.1%). Overall, 65 ACT regimens were administered, most commonly combining IL-1 and TNF inhibitors. Thirty-nine regimens were discontinued because of lack of efficacy, secondary loss of response, or adverse events. At the final follow-up, 26 patients (68%) remained on ACT, with a median treatment duration of 60 months (range, 11-186). CONCLUSIONS: ACT offers significant clinical benefits for patients with difficult-to-treat SAIDs, though challenges such as secondary loss of efficacy and infection risks remain.

Humans↗

Mucocutaneous paraneoplastic manifestations of hematologic malignancies.

PURPOSE: To review the clinical manifestations, pathophysiology, and oncologic implications of the major mucocutaneous paraneoplastic syndromes that can appear in patients with hematologic malignancies. METHODS: A comprehensive search of the medical literature was conducted. RESULTS: In vesiculobullous conditions, although the primary lesions are blisters, observed abnormalities may include large, flaccid bullae (pemphigus vulgaris), superficial crusted erosions (pemphigus foliaceus), or erythema multiforme-like lesions (paraneoplastic pemphigus). Paraneoplastic neutrophilic dermatoses include Sweet's syndrome and pyoderma gangrenosum. In both of these conditions, the skin lesions are characterized by a dermal infiltrate of mature neutrophils. Vascular dermatoses include vasculitis and erythromelagia. Papulosquamous conditions are characterized by small (papules) or large (plaques) raised skin lesions and are usually associated with solid tumors. Amyloidosis is a malignancy-related condition that probably stems from immune dysregulation. RECOMMENDATIONS: Continued surveillance of patients with potential cutaneous paraneoplastic syndromes is necessary, since the malignancy may not be immediately detectable. Some of the cutaneous paraneoplastic syndromes will respond to specific measures, such as systemic corticosteroid therapy, but for the most part, successful resolution requires eradication of the underlying malignancy.

Hematologic Diseases↗

Therapy of scleritis with cytotoxic agents.

Four patients with severe anterior scleritis that had not responded to conventional therapy were treated with cytotoxic immunosuppressive alkylating agents alone (one case) or in combination with prednisone (three cases). Two of the patients had Wegener's granulomatosis, one pyoderma gangrenosum, and one rheumatoid vasculitis. All patients responded well to this therapy with a decrease in inflammation and a healing of the sclera. Other systemic manifestations of the underlying diseases often responded well also.

Adult↗

The dermatopathologic manifestations of hepatitis C infection: a clinical, histological, and molecular assessment of 35 cases.

Cutaneous eruptions related to hepatitis C virus (HCV), a major cause of hepatitis in the setting of blood transfusion, intravenous drug abuse, organ transplantation, and hemodialysis, are typically reported as isolated cases. We encountered 35 cases of HCV infection associated with cutaneous eruptions. The present study evaluates paraffin-embedded, formalin-fixed tissue sections stained with hematoxylin and eosin from biopsy specimens of skin lesions from 35 patients seropositive for HCV. In 20 cases, reverse transcriptase polymerase chain reaction (RT-PCR) was performed using a probe for HCV RNA; the RNA was detected through the action of alkaline phosphatase on the chromogen nitroblue tetrazolium and bromochloroindolyl phosphate. The clinical spectrum comprised dermatomyositis-like photodistributed eruptions, palpable purpura, folliculitis, violaceous and perniotic acral lesions, ulcers, nodules, and urticaria. Lesions were also classified histopathologically by the dominant reaction pattern: vasculopathies of neutrophilic, lymphocytic, and granulomatous vasculitis and pauci-inflammatory subtypes (15 patients); palisading granulomatous inflammation (3 patients); sterile neutrophilic folliculitis (5 patients); dermatitis herpetiformis (1 patient); lobular panniculitis composed of neutrophilic lobular panniculitis in 2 patients and benign cutaneous polyarteritis nodosa in 1 patient; neutrophilic dermatoses, including neutrophilic urticaria, neutrophilic eccrine hidradenitis, and pyoderma gangrenosum (3 patients); interface dermatitis (3 patients); and low-grade lymphoproliferative disease of B-cell lineage representing marginal zone lymphoma in 1 patient and a clonal plasmacellular infiltrate in another patient. In most cases, whereas 1 of the aforementioned disorders defined the dominant reaction pattern, there was an accompanying secondary reaction pattern, defining a hybrid picture. Endothelial changes including endothelial cell enlargement and effaced heterochromatin with margination of the chromatin to the nuclear membrane were seen in several cases; in some cases similar cytopathic changes also involved the supporting pericytes, eccrine ductular cells, or keratinocytes. The RT-PCR analyses in 8 of 20 cases examined revealed HCV RNA expression in a focal, weak fashion in endothelia and perivascular inflammatory cells in those cases showing vasculopathic changes. Viral parasitism of endothelia may be important in cutaneous lesional propagation in the setting of HCV infection. Cross-reactivity between endogenous and viral antigens, leading to cellular and/or type II immune reactions; viral tropism to B lymphocytes, resulting in B cell expansion with resultant autoantibody production; and circulating immune complexes containing monoclonal cryoglobulins may also be of pathogenetic importance. Tropism of the virus to B lymphocytes provides a mechanism for the development of low-grade clonal B cell lymphoproliferative disease in this setting.

Adult↗

[Mycosis fungoides presenting as annular erythema].

INTRODUCTION: Mycosis fungoides is a lymphoma, the classical clinical form of which involves erythematosquamous lesions. However, it can present various atypical aspects: hyper pigmentation or hypo pigmentation, suggestive of pyoderma gangrenosum or ichtyosis. We report a case of mycosis fungoides, unusual in its presentation in the form of centrifugal annular erythema. OBSERVATION: A 78 year-old man had developed a parapsoriasis in plaques for more than 20 years. In May 2002 he consulted because of the recent infiltration of one of the plaques, without concomitant pruritus. The clinical examination revealed 3 lesions of the popliteal groove of the right groin and the left cheek suggestive of centrifugal annular erythema. Histology, revealing Pautrier microabscesses, was compatible with the diagnosis of mycosis fungoides. Evolution was marked by the spontaneous regression of the plaque on the face and remission of the other two plaques after local treatment with chloromethin and topical corticosteroids. Nevertheless, new plaques appeared despite continued treatment, combined with PUVA therapy sessions. DISCUSSION: When searching the literature, we only found one other case of mycosis fungoides, the clinical aspect of which was a centrifugal annular erythema, but in which the histological examination confirmed the diagnosis of mycosis fungoides. Our case report is also unusual in the clinical regression of the lesion on the face, without treatment; this has only been reported in two cases. Mycosis fungoides can appear in various clinical forms. The centrifugal annular erythema form is rare, but this diagnosis should be evoked.

Adrenal Cortex Hormones↗

[Nocardia brasiliensis leg ulcer and nodular lymphangitis in France].

INTRODUCTION: Nocardia brasiliensis is a very rarely reported cause of chronic phagedenic ulcerations. We report the case of an elderly woman who developed such an infection after falling on her right leg on the road in the Bresse country (an essentially agricultural and bovine-cattle breading region) and developed a chronic phagedenic ulcer secondarily complicated by nodular lymphangitis of the thigh. CASE REPORT: A 75 year-old woman fell on her right leg on the side of the main road outside her hamlet in the Bresse country and secondarily developed a chronique phagedenic ulceration. We first considered her as suffering from pyoderma gangrenosum. A complete scanning only revealed an autoimmune thyroiditis and a rapidly healing gastric ulceration, and none of the treatments, either local or systemic, helped the skin condition to heal. After 3 weeks of application of a local corticoid ointment, the patient developed fever, general malaise, an exacerbation of her wound and an infiltration of the skin round her knee, together with nodular lymphangitic dissemination. A supplementary bacterial swab disclosed massive proliferation of a slow-growing Gram-positive bacillus, which proved to be Nocardia brasiliensis, together with a methicillino-sensitive Staphylococcus aureus. The treatment with sulfamethoxazole-trimetoprim gave a rash after 12 hours and was changed to amoxicillin and clavulanic acid, which rapidly proved to be permanently effective. DISCUSSION: The revelation of this particular slow-growing bacteria is difficult and requires bacterial swabs. Nocardia brasiliensis is relatively rare in primary skin ulcerations and we discuss the reasons why an elderly women should find this bacteria on the road outside her hamlet in the French countryside. This particular infectious condition requires general scanning, to make sure that the primary skin condition does not extend to other organs. We review the therapeutical options for patients who exhibit allergic reactions to the classically effective antibiotic drugs.

Accidental Falls↗

Subcutaneous abscesses in a patient with ulcerative colitis.

Ulcerative colitis (UC) is a clinical form of inflammatory bowel disease. The association of pyoderma gangrenosum or erythema nodosum with UC is well known. In addition, pustular eruption has been reported in UC. We describe a patient with UC who exhibited subcutaneous abscesses, as well as pustular eruption with a clinical course paralleling that of UC exacerbation.

Abscess↗

Treatment of psoriatic arthritis with granulocyte and monocyte adsorption apheresis.

Granulocyte and monocyte adsorption apheresis (GCAP) is a new extracorporeal apheresis treatment modality that removes pathogenic granulocytes. Recently, we found that GCAP is useful for treating pyoderma gangrenosum and pustular psoriasis. We thought that this treatment may also be effective for treating other disorders attributable to activated granulocytes and studied the efficacy of GCAP in 4 patients with psoriatic arthritis. Treatment with GCAP resulted in remarkable clearing of joint pain, suggesting that GCAP is valuable for treating arthritis as well as skin disorders. We present a detailed description of these patients and this novel therapy.

Adult↗

Cutaneous manifestations of Crohn's disease.

Crohn's disease is a chronic inflammatory bowel disease which may have distinctive mucocutaneous manifestations. Included in this group are perianal, peristomal, and perifistular ulceration, as well as granulomatous cutaneous inflammation separate from gastrointestinal tract openings (metastatic Crohn's disease). In the oral cavity, both ulcerations and granulomatous nodules may occur. Malabsorption of nutrients may lead to several changes, including an acrodermatitis enteropathica-like syndrome secondary to zinc deficiency. Patients with Crohn's disease may also have pyoderma gangrenosum, erythema nodosum, cutaneous vasculitis, and other less specific changes.

Crohn Disease↗