[Pyoderma vegetans--pyoderma gangraenosum].
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Pyoderma was studied among a representative sample of the residents of four remote Amerindian villages, Amazonas State, Brazil, during July-August 1976. The overall prevalence among the 775 inhabitants examined was 11%, with little intervillage variation. When the attack rates for the entire sample population were calculated by 5-year age intervals, the 0- to 4-year-olds had the highest rate, 31%. The highest prevalence, 38%, was found among 3-year-olds. Attack rates were not apparently related to sex. Cultures which were taken from representative pyoderma lesions from people in the four survey villages and from three additional villages were studied by a modified delayed culture technique for recovery of gram-positive pathogens from silica-gel desiccated swabs. Group A and group G B-hemolytic streptococci, coagulase positive Staphylococcus aureus, and Corynebacterium diphtheriae were isolated. Group A S. pyogenes was most commonly found, occasionally as the sole pathogenic species. No nephritogenic M-types were found, although most isolates were not M-typable. The T-types found corresponded to those previously reported as being pyoderma-associated. Most pyoderma-associated C. diphtheriae isolates were non-toxigenic. Biotypes gravis and mitis were equally represented.
Seven patients with blastomycosis-like pyoderma had skin lesions of four months' to six years' duration. The criteria for the diagnosis of blastomycosis-like pyoderma include the clinical presentation of large verrucous plaques with multiple pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically, and the growth of at least one pathogenic bacteria from the culture of a tissue-biopsy specimen. The differential diagnosis includes deep fungus infection (especially North American blastomycosis), bromoderma, pyoderma gangrenosum, mycobacterial infections, giant keratoacanthoma, and squamous cell carcinoma. Generally, the patients had one or more conditions that could have affected their systemic or local immunologic competence to infection. We believe that the clinical and histologic features in these cases of blastomycosis-like pyoderma were produced by an unusual, exaggerated, vegetating-tissue reaction to a primary or secondary bacterial infection.
The authors report three personal cases of phagedenic pyoderma associated with hemopathy. Studying twenty-five other cases, described in the reviews, the question can be debated on three levels: 1) The clinical characteristics of hemopathic pyoderma remain non-specific. However blisters, pustules, and even vegetating lesions occur very often; 2) the etiology of hemopathy is subject to change; acute leukaemia or myeloproliferation syndrome. During the polyglobulars associated with pyoderma, anemia and myelofibrosis appear quite constantly; 3) finally on the pathogenic level, the recent works tend towards the hypothesis of a damage in the functioning of the polymorphonuclears hence displaying the increase in the inflammation during phagedenic pyoderma.
Studies of the epidemiology of acute glomerulonephritis (AGN) following pyoderma reported over the past 15 years have been reviewed. Investigations in Alabama, at Red Lake in Minnesota, and in Trinidad proved of special interest because they contribute new information concerning the natural history of streptococcal skin infections and the role of such infections in AGN. Interesting contrasts between streptococcal infections of the skin and those of the throat are now apparent. Compared with pharyngeal infections, skin infections are more common in young preschool children, are caused by different serotypes, and differ in the nature of the streptococcal antibody response. A number of new M-serotypes of group A streptococci, including several of importance in AGN, were found in studies of pyoderma. In contrast to M-types 1 and 12 (those of major importance in AGN followng pharyngitis), M-types 2, 49, 55, 57, and 60 are now recognized to be of major importance in AGN following pyoderma. Although streptococcal skin infections are quire important in AGN, they do not result in acute rheumatic fever.
A 67-year-old woman suffered from an ovarian carcinoma with lymph nodes metastasis. During 3 years, she was treated with alkylating agents (Melphalan). At the end of therapy, no recurrence was observed. Two years later, she developed concomitantly pyoderma gangrenosum and acute myelomonocytic leukaemia. Death occurred rapidly. The association between pyoderma gangrenosum and acute leukaemia is discussed in the light of 16 cases previously reported in the literature. In this case, an induction of leukaemia by cytostatic drugs seems likely. The authors conclude that pyoderma gangrenosum may be considered as a cutaneous signs of acute leukaemia.
Two patients with pyoderma gangrenosum have responded remarkably well to treatment with Clofazimine (Lamprène). The first patient, a 68-year old women suffered from pyoderma gangrenosum of the buttock and left leg and on the incision scar for cancer of the breast. Laboratory findings showed monoclonal dysglobulinemia (alpha 2-kappa 2). A daily dose of 300 mg of Clofazimine resulted in complete healing with ten days. The second patient was a 24-year old women suffering from ulcerative colitis and a rapidly progressing pyoderma gangrenosum of the left leg. The lesions was completely healed after two weeks of Clofazimine therapy. The dosage was 200 mg daily and was increased to 400 mg daily. Our cases showed decreased cellular immunity and their phagocytic activity was variable.
Pyoderma gangrenosum is an ulcerative skin disorder with typical clinical characteristics. Histologic and laboratory findings are nonspecific. Pyoderma gangrenosum is associated with internal disorders including inflammatory bowel disease, paraproteinemias, leukemias, and arthritis. The pathogenesis of pyoderma gangrenosum is unknown, although a partial defect of cell-mediated immunity may exist. Treatment includes bedrest, local care, sulfonamides, sulfones, and corticosteroids.
Since 1968 we have been treating a patient, who has had a combination of pyoderma gangraenosum (dermatitis ulcerosa) and signs that may indicate early multiple myeloma. She also had carcinoma of the colon, which was successfully operated. The pyoderma healed later after intensive and successful cytostatic treatment of the "myeloma". The ulcers remain practically healed and the protein pattern is normal in May 1977. Such cases are rare and a search in the literature has not been very rewarding. In our own series of more than 200 cases with myeloma this combination is unique. The lieterature is discussed in detail with data on the follow-up on some of the patients.
A patient is described who developed multiple areas of inflammatory pyoderma on the face, leading to extensive ulceration. Repeated investigations failed to demonstrate any specific bacteria or fungus responsible and trials of treatment with various antibiotics proved unsuccessful. Histology revealed a granulomatous abscess-like lesion, and the condition resembled what has been described as malignant pyoderma. The patient was successfully treated with oral dapsone and intralesional steroids.
Two patients developed persistent ulcers on the trunk after cutaneous surgery. Both had "chemical" diabetes mellitus. Bacteriologic and histopathologic studies of the ulcers were not revealing of cause. The characteristics of the ulcers are described, and are contrasted with typical lesions of pyoderma gangrenosum and Meleney's postoperative progressive synergistic bacterial gangrene. We believe these patients had variant lesions of pyoderma gangrenosum.
Herpes simplex virus type 2 was isolated and identified from the vesicular border of pyoderma gangrenosum lesions on the genital region of a patient with chronic lymphatic leukemia. Dramatic relief of pain as well as quick disappearance of the vesicular margin of the lesions and of the inflammatory halo around them occurred as a result of local treatment with a solution of zinc acetate. A careful search for a viral agent should be done in every case of pyoderma gangrenosum occurring in a patient with a hematological malignancy or/and impaired immunity, especially if the lesions are on the face or in the genital region.
Case history of a 68 years old patient with pyoderma gangrenosum and IgG-paraproteinemia with kappa type light chains. Paraproteins which are observed in pyoderma gangrenosum without overt myeloma could represent early symptoms of plasmocytoma.
Chronic myeloid leukaemia was observed 20 months after onset of pyoderma gangraenosum in a 35-year-old female. In this case an induction by cytostatic drugs could be excluded, confirming the association of pyoderma gangraenosum with leukaemias.
Host defense mechanisms were studied in a patient with recurrent pyoderma of the scalp. Evaluation of the patient's inflammatory response demonstrated normal yeast phagocytosis, normal capillary tube migration, normal results from a nitroblue tetrazolium dye test, and significantly decreased neutrophil chemotactic response (NC). The impaired NC was associated with a heat labile plasma inhibitor. Chromatography of the patient's and of normal human plasma demonstrated three distinct protein peaks. Chemotactic inhibitory activity was found in the third peak of the patient's plasma but not in the control plasma. Normal in vitro NC was restored when greater than 40% normal human plasma was added to the column fractions that contained the inhibitor. Based on these findings, a subsequent exacerbation of the patient's pyoderma was treated with fresh frozen plasma, and dramatic clinical improvement occurred within 72 hours.
The case is presented of a patient with busulfan (Myleran) treated myeloid leukaemia, who developed bullous pyoderma gangrenosum. Skin symptoms appeared at the time when treatment was discontinued due to signs of bone marrow depression. The pyoderma disappeared following treatment with systemic steroid.
A cases of Pyoderma vegetans attacking only the outer skin of the eyelid and leaving free the conjunctiva is reported. The diagnosis of this rare condition, belonging to the pemphigus diseases, depends on clinical course and especially on histological investigation. The aetiology of Pyoderma vegetans still remains unknown, an immun-pathological mechanism is discussed.
A 34-year-old man with chronic active hepatitis and pyoderma gangrenosum demonstrated abnormalities in neutrophil and monocyte function. Monocytes from this man had diminished chemotaxis and bacterial phagocytosis. These functions were significantly improved in vitro after one hour of incubation of monocytes with 10(-5) hydrocortisone. Neutrophil function was nornal for bactericidal activity and phagocytosis, but neutrophil chemotaxis was diminished. A plasma inhibitor was not found to explain these phagocyte alterations.