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Results for “Pyoderma Gangrenosum”

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PubMed1976

[Chronic vegetating pyodermia in cellular immune deficiency].

The simultaneous occurrence of pyoderma gangrenosum together with other diseases, most frequently ulcerative colitis, and with disorders of the humoral immune system is known. The etiology of the individual case remains nevertheless unclear in most instances. We report on an otherwise normal patient with normal immunoglobulin levels, who showed, by use of lymphocyte differentiation and functional tests, a nearly complete lack of T-cell reactivity. A substitution with transfusions of fresh blood lead to rapid healing of the clinical picture, which so far had been resistant to long term application of antibiotics, azathioprine and topical steroids. Therefore it is recommended to look for functional defects of the T-cell system as a cause of pyoderma gangrenosum.

Aged↗
PubMed2002

Granulomatous and suppurative dermatitis at interferon alfa injection sites: report of 2 cases.

It has previously been reported that interferon alfa injection sites may develop pyoderma gangrenosum, interface dermatitis, vasculitis, or, more commonly, ulcers characterized by intravascular thrombi and a mixed inflammatory cell infiltrate. We describe 2 patients in whom granulomatous and suppurative dermatitis developed at interferon alfa injection sites. These cases extend the spectrum of interferon alfa injection site reactions. The histologic and clinical similarities of these cases with pyoderma gangrenosum and cutaneous Crohn's disease are explored.

Granuloma↗
PubMed2002

A study of musculoskeletal manifestations in 12 patients with SAPHO syndrome.

Synovium and synovial fluid findings in SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome have not been well characterized, and only a few patients have been described in the Americas. We describe clinical, pathologic, and synovial fluid findings in 12 patients with the SAPHO syndrome: hidradenitis suppurativa (7), acne fulminans or conglobata (3), acneiform folliculitis (1) and palmoplantar pustulosis (1). Routine synovial fluid studies were performed in 6 patients, and light and transmission electron microscopic studies were performed in synovium in 2 patients and in bone in 1. The most common musculoskeletal manifestations included erosive or non-erosive oligoarthritis involving metacarpal phalangeal (MCP) and metatarsal phalangeal (MTP) joints as seen in 9 patients, sclerosis of the sacroiliac joints as seen in 5 patients, and osteitis pubis as seen in 1. Three patients had signs of skeletal hyperostosis. The patients with acne fulminans and acneiform folliculitis had chronic aseptic multifocal osteomyelitis. Synovial fluid was sterile in 7, mildly inflammatory in 5, and highly inflammatory in 2. Electron microscopic studies of synovium in 2 patients and of bone in 1 were not useful to detect microorganisms. Three African-American patients with hidradenitis suppurativa presented with pyoderma gangrenosum, and 2 of them had leukocytoclastic vasculitis, and a life threatening course unresponsive to antibiotics, corticosteroids and immunosuppressive therapy. SAPHO in the Americas is most severe in African-Americans with hidradenitis suppurativa, and it presents with heterogeneous musculoskeletal and cutaneous manifestations including erosive polyarthritis or oligoarthritis with nonspecific mild inflammatory fluid. Leukocytoclastic vasculitis and recalcitrant pyoderma gangrenosum were seen in 2 of our patients with the most severe hidradenitis suppurativa. SAPHO syndrome may present with clinical manifestations similar to those seen with seronegative spondyloarthropathies, but it has distinctive cutaneous, radiographic articular, and bone manifestations. Sites of chronic infection need aggressive antibiotic therapy and may need surgical resection.

Journal Article↗
PubMed2004

Neutrophilic dermatosis of the hands: four new cases and review of the literature.

BACKGROUND: Isolated or predominantly hand involvement in Sweet's syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweet's syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy. METHODS: We report four cases of neutrophilic dermatosis affecting the hands and a review of the literature for similar cases. RESULTS: Of the four patients presented, two had associated carcinomas and one had myelodysplasia in transition to leukemia. The cutaneous symptoms preceded the finding of an occult malignancy. Thirty-two reported cases of neutrophilic dermatoses presenting on the hands showed clinicopathologic features similar to those in our series. Taken together, the mean age at diagnosis was 60.5 years, and 58% of the patients were female. Twenty-five per cent (nine patients) also had myelodysplasia or leukemia, 14% (five patients) ulcerative colitis, 6% (two patients) carcinoma, 6% (two patients) Crohn's disease, and 6% (two patients) seropositive arthritis. CONCLUSIONS: These cases illustrate the importance of recognizing that neutrophilic dermatoses may present uniquely or predominantly on the hands. This presentation is distinctive, and prompt diagnosis may prevent unnecessary medical or surgical therapy and may lead to the earlier diagnosis and treatment of an associated malignancy or other systemic disorder.

Aged↗
PubMed2004

Review article: skin complications associated with inflammatory bowel disease.

Cutaneous manifestations are well-recognized complications of Crohn's disease and ulcerative colitis. The incidence of these manifestations varies widely but, at the time of diagnosis, the mean incidence is around 10%. During the course of the disease, a great variety of skin lesions may develop, many of which are secondary to granulomatous cutaneous disease, reactive skin eruptions, nutritional deficiency and other associated conditions. The disorders that are directly related to the inflammatory process of Crohn's disease include perianal and peristomal ulcers and fistulae, metastatic Crohn's disease and oral granulomatous lesions. Histologically, the features are similar to those found in the inflamed bowel. These lesions usually respond to treatment of the underlying intestinal disease. The most common forms of reactive skin eruption are erythema nodosum and pyoderma gangrenosum. Certain subsets of patients are more susceptible to the development of erythema nodosum; in a previous report from our group, erythema nodosum was seen mainly in females, and in patients with colonic involvement and/or arthritis. This manifestation tends to occur during the first 2 years of the clinical course of the disease and may recur in approximately one-half of cases. Infliximab is highly effective in healing refractory lesions of erythema nodosum and pyoderma gangrenosum. Manifestations that are secondary to nutritional deficiency or associated conditions include acrodermatitis enteropathica, psoriasis and autoimmune disorders. For most of the cutaneous manifestations, the primary therapeutic target remains the bowel. Early aggressive therapy can minimize severe complications and maintenance treatment may prevent some devastating consequences.

Colitis, Ulcerative↗
PubMed2000

Elafin is induced in epidermis in skin disorders with dermal neutrophilic infiltration: interleukin-1 beta and tumour necrosis factor-alpha stimulate its secretion in vitro.

BACKGROUND: Elafin, an elastase inhibitor produced by keratinocytes, is overexpressed in the subcorneal region of skin affected by psoriasis, a major feature of which is epidermal infiltration by neutrophil leucocytes. OBJECTIVES: We studied the expression of elafin in the epidermis in other skin disorders characterized by dermal neutrophil infiltration and in skin disorders with dermal lymphocyte infiltration. PATIENTS/METHODS: We examined biopsies from the lesional skin of patients with Behçet's syndrome, Sweet's syndrome, pyoderma gangrenosum, cutaneous allergic vasculitis and acute bacterial infection (cellulitis), and from the skin of patients with chronic prurigo, discoid lupus erythematosus and psoriasis. We performed in vitro experiments using cultured keratinocytes treated with mediators such as interleukin (IL)-1 beta, tumour necrosis factor (TNF)-alpha, IL-6, neutrophil elastase and interferon (IFN)-gamma. RESULTS: Anti-elafin antibody showed a strong reaction with the subcorneal region of the epidermis in patients with Behçet's syndrome, Sweet's syndrome, pyoderma gangrenosum, cutaneous allergic vasculitis and acute bacterial infection (cellulitis), but showed no reaction in skin from patients with dermal lymphocyte infiltration such as is seen in chronic prurigo and discoid lupus erythematosus. The in vitro experiments demonstrated that treatment with IL-1 beta and TNF-alpha resulted in 2.6-fold and 4-fold stimulation of elafin secretion, respectively, whereas IL-6, neutrophil elastase and IFN-gamma caused no significant changes in elafin release. CONCLUSIONS: These results suggest that inflammatory mediators such as IL-1 beta or TNF-alpha secreted by dermal neutrophils may be involved in overexpression of elafin in keratinocytes; this could protect the epidermis from degradation by dermal neutrophil infiltration.

Cell Culture Techniques↗
PubMedDate not supplied

Evaluation of circulating immune complexes in cutaneous diseases associated with immune disorders.

Several skin diseases associated with immune disorders may be related to the formation of circulating immune complexes (CIC) and their skin deposition. Sera from 20 controls and 108 patients, including: 23 psoriasis, 10 lichen planus, 30 atopic eczemas, 32 cases of leprosy, 10 vasculitis and 3 pyoderma gangrenosum were evaluated for the presence of IgG-containing CIC by the microconsumption complement test (MCT). Additionally, the presence of IgE-containing CIC by means of a polyethylene glycol precipitation and radioimmunoassay technique was evaluated in 10 patients with atopic eczema. It was found that 56.5% of psoriatic patients show moderate CIC concentrations, as well as 34% of leprosy patients, with increased levels when bacillus were detected in skin lesions, and in 90% of leukocytoclastic vasculitis. A close relationship between CIC levels and the clinical evolution of skin lesions was demonstrated in patients with pyoderma gangrenosum and lichen planus. IgG-CIC were detected in 33% and IgE-CIC in 30% of patients with atopic eczema, with the presence of both types of CIC in 2 out of 3 cases. The systematic research on CIC presence in some selected skin diseases shows that immune complexes take part, with different degrees of relevance, in the pathogenesis of all the entities.

Adolescent↗
PubMed1997

Malignant pyodermas revisited.

The concept of malignant pyoderma (MP) has created controversy since its origin. The distinction of this disease from pyoderma gangrenosum was based on clinical criteria and response to treatment. Herein we discuss our current ideas on this entity and its possible relationship to Wegener's granulomatosis (WG). Follow-up data from the three original cases of MP are reported, as well as additional clinical and laboratory data from cases subsequently thought to represent MP. Many of these cases have similar clinical features such as facial and periauricular ulceration and occasionally signs or symptoms of WG, including positive titers of antineutrophil cytoplasmic antibodies (with a diffuse cytoplasmic staining pattern) (cANCA). MP represents a distinctive clinical disorder and may be a dermal manifestation of WG. Some cases of MP may represent pyoderma gangrenosum or other undefined systemic illnesses. Such cases of WG can be distinguished on the basis of clinical, histopathologic, and laboratory evidence including cANCA titers. MP should no longer be used as a final clinical diagnosis.

Adult↗
PubMedDate not supplied

[Superficial granulomatous pyoderma].

INTRODUCTION: Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum, which is often diagnosed at a late stage because of misleading clinical-histopathological features. We report a new case of this rare disease. CASE REPORT: A 67 year-old man presented with inflammatory lesions that had become ulcerated over the past 4 months despite prolonged antibiotic therapy. The histopathological aspect was initially suggestive of palisading granuloma annulare and subsequently a necrotizing granuloma compatible with a mycobacteriosis. The various supplementary examinations eliminated the possibility of an infectious or systemic disease. The lesions became painful and ulcerated. A second biopsy showed a neutrophilic dermatosis with dermal necrosis and a granuloma leading to the diagnosis of superficial granulomatous pyoderma. The lesions healed after 3 months of systemic corticosteroids at the dose of 1 mg/kg/day. DISCUSSION: The clinical features of superficial granulomatous pyoderma are similar to those of classical pyoderma gangrenosum. However, its histological profile is more superficial and is associated with dermal granulomas. The latter are unusual in neutrophilic dermatosis and would suggest an infectious disease. This explains the frequent diagnostic errors, with inappropriate anti-infectious treatments and notably unnecessary surgical exeresis.

Adrenal Cortex Hormones↗
PubMed2003

Cutaneous manifestations of Crohn's disease, its spectrum, and its pathogenesis: intracellular consensus bacterial 16S rRNA is associated with the gastrointestinal but not the cutaneous manifestations of Crohn's disease.

The classic pathology of skin disease discontinuous from the inflamed gastrointestinal (GI) tract in patients with Crohn's disease (CD) includes pyoderma gangrenosum (PG), erythema nodosum (EN), and so-called metastatic Crohn's disease. The purpose of this study was two-fold: First, we explored the full spectrum of cutaneous lesions associated with Crohn's disease, and second, we sought to explore a potential molecular basis of the skin lesions in patients with CD. In this regard, we analyzed skin and GI tract biopsies from affected patients for the consensus bacterial SrRNA to determine whether direct bacterial infection was associated with either condition. Formalin-fixed, paraffin-embedded sections were studied and correlated to clinical presentation and histories from 33 patients with CD. Consensus bacterial RNA sequences were analyzed using an RT in situ PCR assay on both skin biopsy and GI biopsy material. The GI tract material included biopsies from 3 patients who had skin lesions and from 7 patients in whom there were no known skin manifestations. There were 8 cases of neutrophilic dominant dermal infiltrates, including pyoderma gangrenosum, 6 cases of granuloma annulare/necrobiosis lipoidica-like lesions, 5 cases of sterile neutrophilic folliculitis, 5 cases of panniculitis, 4 cases of vasculitis, 2 cases of psoriasis, 2 cases of lichenoid and granulomatous inflammation, and 1 case of classic metastatic CD. Intracellular bacterial 16S rRNA was detected in 8 of 10 tissues of active CD in the GI tract, of which 3 of the cases tested were from patients who also developed skin lesions at some point in their clinical course; in contrast, none of the skin biopsies had detectable bacterial RNA. The dermatopathological manifestations of CD discontiguous from the involved GI tract mucosa have in common a vascular injury syndrome, typically with a prominent extravascular neutrophilic and/or histiocytic dermal infiltrate. In addition, this study, the first to document in situ intracellular consensus bacterial SrRNA in the GI tract in CD, suggests that hematogenous dissemination of viable microbes is not associated with the cutaneous manifestations of this disease. Bacteria do, however, appear to play a role in bowel lesions of patients with CD.

Adolescent↗
PubMed1993

Cutaneous vasculitis and other neutrophilic dermatoses.

During 1991 and 1992 there was an abundance of articles dealing with vasculitis and its cutaneous manifestations, as well as with the perhaps related disorders known as neutrophilic dermatoses. This brief review will deal with some of the more controversial areas. The specific issues discussed are those relating to the "new" American College of Rheumatology classification system and its application to patients who have skin lesions of vasculitis, the recently associated diseases, and the newer therapies for vasculitis. In addition, recent publications regarding acute neutrophilic dermatosis (Sweet's syndrome) and pyoderma gangrenosum are briefly discussed.

Humans↗
PubMedDate not supplied

A difficult case of pyrexia.

We present a case of non-specific vasculitis presenting as a pyrexia of unknown origin to illustrate the diagnostic difficulty which can arise with this condition. The diagnostic event was central retinal artery occlusion and the disease was also associated with pyoderma gangrenosum. History, examination, investigations and management are discussed.

Aged↗
PubMedDate not supplied

Cutaneous manifestations of internal diseases.

Collaboration between specialists is necessary for good practice of medicine. The dermatological examination, mainly based on visual characteristics, can greatly help other specialisms when confronted with a seemingly futile skin anomaly. We discuss the various cutaneous features of some metabolic and infectious diseases, the pitfalls in diagnosing lupus erythematosus without knowledge of skin disease and the relationship of dermatological diseases as cutaneous vasculitis, pyoderma gangrenosum, Sweet's syndrome, pemphigus and more to internal disease.

Aged↗
PubMed2002

Abdominal wall mucormycosis successfully treated with amphotericin and itraconazole.

The routine management of cutaneous mucormycosis consists of combined surgical and medical therapy. We present a case of successful medical therapy with sequential liposomal amphotericin B and itraconazole for a peri-ostomal cutaneous Rhizopus infection in a 59-year-old woman following treatment for pyoderma gangrenosum in the setting of inflammatory bowel disease.

Abdominal Muscles↗
PubMed2001

Neutrophilic myositis as an extracutaneous manifestation of neutrophilic dermatosis.

Neutrophilic dermatoses are characterized histologically by a sterile infiltration of neutrophils throughout the dermis. The condition may affect various internal organs, notably the lungs, the digestive tract, and the joints, whereas muscle impairment is considered to be extremely rare. We describe the case of a patient with pyoderma gangrenosum in whom severe sterile neutrophilic myositis developed as the first manifestation of an acute myelogenous leukemia.

Aged↗
PubMed1998

Pyostomatitis vegetans in childhood.

UNLABELLED: Pyostomatitis vegetans is an oral eruption, characterized by small pustules, ulcers and erythematous vegetations of the labial and buccal mucosae as well as labial-attached gingivae. Its importance lies in its high correlation with inflammatory bowel disease. It is commonly associated with skin and inflammatory bowel disease and is rare in children. We here report a sister and brother with onset of the disease at the age of 5 and 7 years, respectively. It is the first report of familial pyostomatitis vegetans occurring in the youngest patients hitherto reported. CONCLUSION: The observation of two sibs with pyostomatitis, vegetans pyoderma gangrenosum and inflammatory bowel disease suggest a hereditary disposition to this rare triad.

Adolescent↗
PubMed2003

Recurrent leg ulcers in a 16-year old Nigerian girl.

This is a report of a 16-year old Nigerian girl who presented with recurrent leg ulcers associated with gastrointestinal symptoms. The management of this patient, who has ulcerative colitis manifesting with pyoderma gangrenosum, underlines the need for thorough evaluation of cutaneous ulcers in the tropics. The medical treatment of ulcerative colitis in childhood, with reservation of bowel resection for complications is preferred.

Adolescent↗