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PubMed1997

Neutrophilic dermatoses: pyoderma gangrenosum and Sweet's syndrome.

Pyoderma gangrenosum and Sweet's syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with little evidence of a primary vasculitis. They share several characteristics and respond to immunosuppressives. Aetiology is felt to represent a manifestation of altered immunologic reactivity. Patients with both conditions concurrently have been described. Diagnosis is based on clinical and histopathological findings. However, clinically the typical forms of the two conditions are quite distinct: pyoderma showing cutaneous ulceration with a purple undermined border and Sweet's syndrome having tender, erythematous, nonulcerated plaques and nodules. Approximately 50% of cases of pyoderma are associated with a specific systemic disorder. These include inflammatory bowel disease, rheumatoid arthritis, non-Hodgkin's lymphoma and myeloproliferative disorders. Many associations with Sweet's syndrome have been described, including acute myeloid leukaemia, myeloma and adenocarcinomas, and haematological malignancy. There is overlap between the two conditions with lesions categorised as Sweet's syndrome being clinically more characteristic of atypical pyoderma and vice versa. We believe that pyoderma and Sweet's syndrome represent a continuum of spectrum of disease. The reason for the clinical differences between the conditions is unclear and merits further investigation but may be explained by varying levels of intensity and extent of the inflammatory process. This review will describe the pathogenesis, clinical features, diagnosis, associations and treatment of the two conditions.

Aged↗
PubMed1998

Pyoderma gangrenosum and extensive caval thrombosis associated with the antiphospholipid syndrome--a case report.

Pyoderma gangrenosum is a rare cutaneous disorder frequently associated with systemic diseases. The authors report the case of a 28-year-old man with pyoderma gangrenosum and inferior cava vein syndrome due to thrombosis. The search for pyoderma gangrenosum-associated conditions (gastrointestinal, rheumatic, neoplasic, and infectious diseases) was negative. A prolonged partial thromboplastin time and a positive test for circulating anticardiolipin antibodies strongly suggested the diagnosis of the antiphospholipid syndrome. There are reports of at least three other cases of pyoderma gangrenosum associated with the antiphospholipid syndrome. This clinical case adds further evidence to the possible link between these disorders and warrants a search for anticardiolipin antibodies in patients with pyoderma gangrenosum.

Adult↗
PubMed2002

Clinical management of pyoderma gangrenosum.

Pyoderma gangrenosum is a noninfectious neutrophilic dermatosis that usually starts with sterile pustules which rapidly progress to painful ulcers of variable depth and size with undermined violaceous borders. In 17 to 74% of cases, pyoderma gangrenosum is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatological or hematological disease or malignancy. Diagnosis of pyoderma gangrenosum is based on a history of an underlying disease, typical clinical presentation and histopathology, and exclusion of other diseases that would lead to a similar appearance. Randomized, double-blinded prospective multicenter trials investigating the treatment of pyoderma gangrenosum are not available. The treatments with the best clinical evidence are systemic corticosteroids (in the initial phase usually 100 to 200 mg/day) and cyclosporine (mainly as a maintenance treatment). Combinations of corticosteroids with cytotoxic drugs such as azathioprine, cyclophosphamide or chlorambucil are used in patients with disease that is resistant to corticosteroids. The combination of corticosteroids with sulfa drugs, such as dapsone, or clofazimine, minocycline and thalidomide, has been used as a corticosteroid-sparing alternative. Limited experience has been documented with methotrexate, colchicine, nicotine, and mycophenolate mofetil, among other drugs. Alternative treatments include local application of granulocyte-macrophage colony-stimulating factor, intravenous immunoglobulins and plasmapheresis. Skin transplants (split-skin grafts or autologous keratinocyte grafts) and the application of bioengineered skin is useful in selected cases in conjunction with immunosuppression. Topical therapy with modern wound dressings is useful to minimize pain and the high risk of secondary infection. The application of topical antibacterials cannot be recommended because of their potential to sensitize and their questionable efficacy, but systemic antibacterial therapy is mandatory when infection is present. Despite recent advances in therapy, the prognosis of pyoderma gangrenosum remains unpredictable.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Prevalence of methicillin-resistant Staphylococcus aureus (MRSA) in community-acquired primary pyoderma.

BACKGROUND: Although prevalence of MRSA strains is reported to be increasing, there are no studies of their prevalence in community-acquired primary pyodermas in western India. AIMS: This study aimed at determining the prevalence of MRSA infection in community-acquired primary pyodermas. METHODS: Open, prospective survey carried out in a tertiary care hospital in Mumbai. MATERIALS AND METHODS: Eighty-six patients with primary pyoderma, visiting the dermatology outpatient, were studied clinically and microbiologically. Sensitivity testing was done for vancomycin, sisomycin, gentamicin, framycetin, erythromycin, methicillin, cefazolin, cefuroxime, penicillin G and ciprofloxacin. Phage typing was done for MRSA positive strains. RESULTS: The culture positivity rate was 83.7%. Staphylococcus aureus was isolated in all cases except two. Barring one, all strains of Staphylococcus were sensitive to methicillin. CONCLUSIONS: Methicillin resistance is uncommon in community-acquired primary pyodermas in Mumbai. Treatment with antibacterials active against MRSA is probably unwarranted for community-acquired primary pyodermas.

Adolescent↗
PubMed2002

[Pregnancy: a possible etiology of pyoderma gangrenosum. A case report and review of the literature].

OBJECTIVE: We report an unusual case of pyoderma gangrenosum at 26 weeks gestation and review other cases reported in the literature in order to examine the underlying causes and determine the appropriate diagnostic and therapeutic approach to this uncommon skin disease. Patient and methods. The patient developed an acute abdominal syndrome at 26 weeks gestation (laparotomy was non contributive) followed by pyoderma gangrenosum of the abdominal wall. Seven other cases have been reported in the literature associating pyoderma gangrenosum with pregnancy. We analyzed the clinical findings in these cases. RESULTS: Corticosteroid therapy successfully controlled pyoderma gangrenosum in our patient. Pregnancy outcome was favorable with little effect on the newborn. Complete healing was achieved after several months of treatment. CONCLUSION: Histological lesions are nonspecific. Diagnosis of pyoderma gangrenosum is generally based on clinical signs. Systemic disease is associated in half the cases (inflammatory bowel disease, myelodysplastic syndrome, rheumatoid arthritis). The impact on pregnancy appears to be limited. Treatment is based on high-dose systemic corticosteroid therapy or cyclosporin. The two drugs may have to be combined. Search for an associated disease and appropriate treatment is particularly important for the outcome of pregnancy.

Abdominal Wall↗
PubMed1989

[The seasonality of pyoderma in workers of the Don Basin coal mines].

Seasonal patterns of pyoderma have been studied in 2899 miners, as well as the relationship between these patterns and the patients' age, occupation, and the major weather factors. The incidence of pyoderma regularly increases in summer and autumn, whatever the geological conditions in the mines. One peak (summer-autumn) has been determined for the dermatologic forms and 2 (winter and summer-autumn) for the surgical ones. The seasonal fluctuations in pyoderma incidence are more manifest in subjects aged over 50. No close correlation between pyoderma incidence and the major weather factors has been detected. Seasonal cyclic pattern of pyoderma is related to the adaptation of man to the environmental conditions.

Absenteeism↗
PubMed1983

Defective monocyte function in pyoderma gangrenosum with IgG kappa paraproteinaemia.

Peripheral blood monocytes from a patient with pyoderma gangrenosum and paraproteinaemia showed defective phagocytosis; longitudinal observations demonstrated an association between the defect, the level of paraprotein and the clinical activity of the pyoderma. Treatment with Melphalan led to a reduction in the paraprotein level and remission of the pyoderma and was accompanied by normalization of monocyte function. After 8 months remission the paraprotein level rose again and was accompanied by a recurrence of the monocyte defect; shortly after this the pyoderma recurred. Pre-incubation of normal monocytes with the patient's plasma or immunoglobulin fractions revealed that a similar defect could be induced in normal cells by the patient's monomeric IgG. The patient's serum also had anti-heparin activity and the relationship between this and the phagocytic defect was explored. These studies indicate a possible pathogenetic mechanism underlying the association between pyoderma gangrenosum and monoclonal gammopathy.

Aged↗
PubMed1984

Serum thyroxine and triiodothyronine concentrations in canine pyoderma.

In an effort to evaluate the effect of pyoderma on circulating iodothyronines, plasma triiodothyronine (T3) and thyroxine (T4) values were determined before and after thyroid stimulating hormone administration in 25 dogs with pyoderma and in 15 controls. Basal T4 values were increased in dogs with pyoderma, but neither stimulated T4 nor T3 values were altered by this condition. On the basis of low values for circulating iodothyronines, hypothyroidism was suspected in 3 dogs in the pyoderma group. The dog with the most involved lesions had extremely low T3 and T4 values as well as an autoimmune disease. It was concluded that most dogs with pyoderma do not have thyroid dysfunction.

Animals↗
PubMed1982

[Microbiological substantiation of the use of erycycline in the therapy of chronic pyoderma].

Sensitivity to tetracycline, erythromycin and erycycline was studied in 60 strains of Staph. aureus isolated from the foci of chronic pyoderma and nasopharynx of 30 patients suffering from chronic relapsing pyoderma for 2-5 years. The isolates were 2.6 and 3.2 times more sensitive to erycycline than to erythromycin and tetracycline, respectively. This substantiated the advisability of erycycline use in combined therapy of patients with chronic pyoderma. The staphylococcal strains isolated from the nasopharynx were less sensitive to the antibiotics than the strains isolated from the foci of the skin affection which must explain prolonged persistence of staphylococci in the nasopharynx in spite of the antibiotic use in a dose sufficient for elimination of the microorganism affecting the skin. This might be also the cause of pyoderma relapses. It is concluded that the dose of the antibiotic used for sanation of the nasopharynx in patients with chronic pyoderma should be higher than that for elimination of the pustulous process on the skin.

Adult↗
PubMed1995

Antimicrobial susceptibility and rRNA gene restriction patterns among Staphylococcus intermedius from healthy dogs and from dogs suffering from pyoderma or otitis externa.

A total of 60 Staphylococcus intermedius strains from dogs were investigated by their sensitivity to various antibiotics (50 strains) and by their rRNA gene restriction patterns (ribotyping) (60 strains). Fifteen isolates were from healthy dogs, 9 with otitis externa, and 36 with pyoderma, including 10 strains from a previous study. Sixty per cent of the 50 strains tested for antibiotic susceptibility demonstrated resistance to penicillin, 24% to spiramycin, 20% to tetracycline, 16% to chloramphenicol, and 2% to fucidic acid. All isolates were susceptible to amoxycillin with clavulanic acid, enrofloxacin, and sulphonamides with trimethoprim. There were no significant differences in antimicrobial susceptibility patterns observed among isolates from pyoderma, otitis externa or healthy dogs. Among the 60 strains studied by ribotyping, 10 different ribotypes were identified: 6 different ribotypes among isolates from otitis externa, 8 among isolates from pyoderma, and 5 among isolates from healthy dogs. One ribotype (profile C) was dominant among the isolates from healthy dogs while another ribotype (profile A) was dominant among strains from dogs suffering from pyoderma. This profile was not demonstrated in any of the strains from healthy dogs. From 5 different dogs suffering from pyoderma, 2 different clones were demonstrated based on their plasmid profile and antibiogram. In these dogs 1 of the clones always belonged to ribotype A. The results concerning strains of S. intermedius isolated from furunculosis suggest the existence of distinct subpopulations with different pathogenicity to dogs.

Animals↗
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↗
PubMed1982

Pyoderma gangrenosum. A possible cause of wound necrosis.

Pyoderma gangrenosum is a destructive cutaneous disorder characterized by painful, rapidly enlarging ulcers with under-mined bluish and purplish red margins. This disease is most frequently found in association with ulcerative colitis. However, in the last three years, four cases of pyoderma gangrenosum precipitated by surgical procedures have occurred, and only one of these patients had ulcerative colitis. All four patients responded to steroid therapy and local wound care. Pyoderma gangrenosum can first be suspected by recognition of the previously described classic lesion. In addition, the absence of significant microbial growth and the lack of a specific etiology ascertained on routine and special staining of biopsy material is suggestive of the diagnosis. Histologic characteristics are entirely nonspecific but can be helpful in ruling out other specific entities. The progressively destructive nature of this process in the face of what would seem to be appropriate therapy for bacterial wound infection requires that pyoderma gangrenosum, as well as other aspects of the differential diagnosis of a persistent ulcerative lesion, such as fungal infection, factitious ulcer, or vasculitis, be considered.

Adult↗
PubMed1984

Pyoderma gangrenosum at the parastomal site in patients with Crohn's disease.

Pyoderma gangrenosum is a rare idiopathic skin condition often associated with other diseases, including Crohn's disease. We believe this is the first report of cases in which pyoderma gangrenosum was identified at the parastomal site in patients with Crohn's disease. Four episodes of the condition occurred in three patients. In these patients, pyoderma gangrenosum first appeared after two to three surgical procedures and between ten months and five years after the initial surgery. The disease course varied from resolution within two months with corticosteroid therapy to resolution only after revision or relocation of the stoma with resection of recurrent disease segments. Parastomal pyoderma gangrenosum at the ileostomy site in patients with Crohn's disease may be more common than previously thought. Optimal treatment has not yet been established, and treatment needs to be tailored for each patient.

Adult↗
PubMed2001

[Pyoderma gangrenosum: successful topical therapy with tacrolimus (FK506)].

Pyoderma gangrenosum is a distinct clinical entity characterized by chronic, recurring, destructive ulcerations. Although the pathogenesis of pyoderma gangrenosum is unknown, immunologic aberrations of neutrophil granulocytes seem to be important. Systemic steroids and macrolide lactones such as cyclosporin A and tacrolimus have been reported to be useful in the clinical management of disease. Pyoderma gangrenosum has been found to be associated with several systemic diseases. The association with chronic ulcerative colitis is well known, but the diagnosis may be complicated by early administration of systemic steroids. Therefore, local immunosuppression with topically applied agents could be an efficient therapeutic alternative especially for mild or early cutaneous lesions.We describe the successful topical treatment of a patient with multiple lesions of pyoderma gangrenosum with 0,1% tacrolimus (FK506) ointment which is known to have better dermal penetration and higher immunosuppressive potency than topical cyclosporin A. In addition, other indications for topical tacrolimus are discussed.

Abdomen↗
PubMed2002

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.

Female↗
PubMed1987

Pyoderma gangrenosum with myelofibrosis.

Pyoderma gangrenosum is a papulovesicular skin disorder commonly associated with underlying systemic disease, but rarely with the myeloproliferative syndromes. A case of rapidly progressive pyoderma is cited in a 77-year-old white man who had no other evidence of disease aside from macrocytic anemia. Bone marrow biopsy revealed proliferation of fibroblasts and a dense reticulin network consistent with myelofibrosis. Response of the pyoderma to steroid therapy was dramatic. Although pyoderma gangrenosum is more commonly associated with inflammatory bowel disease and rheumatoid arthritis, this is the fifth reported case of its coexistence with idiopathic myelofibrosis.

Aged↗
PubMed1997

A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome.

OBJECTIVE: To describe a multigenerational family with transmission of an autosomal dominant disorder characterized by pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne. MATERIAL AND METHODS: We present a detailed case report of a 39-year-old man with arthritic changes in several joints, pyoderma gangrenosum, and cystic acne. Several relatives from three generations of his family underwent clinical and genetic investigations. The findings in this kindred are reported. RESULTS: Ten affected family members in three generations manifested variable expression of a pauciarticular, nonaxial, destructive, corticosteroid-responsive arthritis that began in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence and beyond. Other less commonly associated features included adult-onset insulin-dependent diabetes mellitus, proteinuria, abscess formation at the site of parenteral injections, and cytopenias attributable to sulfonamide medications. Laboratory evaluation was nondiagnostic. Genetic studies excluded linkage to the major histocompatibility locus. CONCLUSION: The acronym of PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne) is suggested for this newly recognized pleiotropic autosomal dominant disorder. The nature of the genetic alteration in PAPA syndrome is unknown.

Acne Vulgaris↗