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We report a case of atypical bullous pyoderma gangrenosum associated with acute myeloid leukaemia in which we found atypical myeloid cells within the skin lesion. Although there have been many reported cases of leukaemia-associated pyoderma gangrenosum, the finding of myeloblasts in the skin has rarely been described.
A 34-year-old woman who was 20 weeks pregnant developed pyoderma gangrenosum while receiving treatment for infarct pneumonia. Because of her septic condition an abortion was performed. The patient received intravenous prednisolone as a pulse treatment for her pyoderma gangrenosum, which was followed by oral methylprednisolone and oral dapsone for 6 weeks. After dose reduction the patient had a severe relapse. The steroid dose was increased and dapsone was replaced by cyclosporin A. The smaller ulcers healed spontaneously, but skin grafting had to be performed for the large ulcer. The steroid therapy was tapered and discontinued. Cyclosporin A was continued for 10 weeks as monotherapy. Subsequently, the patient was free of symptoms for 7 months. Twelve months after the diagnosis of pyoderma gangrenosum the patient developed acute myeloid leukaemia accompanied by a recurrence of the skin disease.
We present a patient with pyoderma gangrenosum, a rare complication of ulcerative colitis. The patient's disease was limited to the distal colon, was clinically mild and responded quickly to treatment, and yet it was associated with pyoderma gangrenosum and arthritis, complications generally associated with more severe and extensive ulcerative colitis.
Pyoderma gangrenosum is a rare skin disease of unknown pathogenesis associated, in almost 8 out of 10 cases, with a systemic disease, notably enterocolitis or hemopathy. We report the case of a 57-year old man who had been presenting with pyoderma gangrenosum for 5 years when he developed a rheumatoid-like seronegative chronic polyarthritis. The occurrence, some time later, of a supraclavicular adenopathy led to the diagnosis of Hodgkin's disease. To our knowledge, the pyoderma-chronic polyarthritis-Hodgkin's lymphoma association has never been reported. Treatment of the lymphoma resulted in complete disappearance of cutaneous and articular symptoms. The fact that neither the skin disease nor the polyarthritis recurred during a 3-year follow-up after treatment was discontinued, incites us to discuss the possibility that the pyoderma and the polyarthritis observed in this patient were neoplastic diseases.
We report a 55-year-old woman with bullous systemic lupus erythematosus, who later developed pyoderma gangrenosum (PG). Dapsone was effective for the eruption of bullous bullous systemic lupus erythematosus but not for pyoderma gangrenosum. Cyclosporine was effective for the skin lesions of pyoderma gangrenosum. This is the first reported case of PG associated with bullous systemic lupus erythematosus.
A potentially contributing factor to the development and chronicity of pyoderma gangrenosum is infection with the relatively recently characterized human pathogen, Chlamydia pneumoniae. C pneumoniae is an obligate intracellular bacterium that can infect endothelial, monocyte, and smooth muscle cells and is associated with cardiopulmonary diseases. A case of serologically, polymerase chain reaction-positive, immunohistochemically, and culture-documented viable C pneumoniae organisms in a chronic pyoderma gangrenosum ulcer is reported, a finding that has not been described previously.
Pyoderma gangrenosum is an uncommon skin disorder that is associated with ulcerative colitis, Crohn's disease, polyarthritis, and other conditions. Clinically it is usually characterized by painful ulceration of the skin and subcutaneous tissues, predominantly of the lower limbs. Treatment is empirical and often unsatisfactory. We report two cases of pyoderma gangrenosum associated with inflammatory bowel disease successfully treated with topical disodium cromoglycate.
A 36-yr-old patient suffering from chronic ulcerative colitis complicated by severe pyoderma gangrenosum of the leg is presented. Pyoderma gangrenosum rapidly progressed and failed to respond to a combined treatment of bed rest, prednisone, sulfasalazine, povidone-iodine, compresses, and debridement. Pyoderma gangrenosum responded and disappeared with no recurrence during the following months after the combined treatment of methylprednisolone, dapsone, and zinc sulfate.
PURPOSE: To describe the clinical characteristics of periocular pyoderma gangrenosum and to highlight features that may encourage early diagnosis of this extremely rare condition. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS AND METHODS: Four patients with periocular pyoderma gangrenosum were treated in the Orbital Unit at Moorfields Eye Hospital over the course of a decade. MAIN OUTCOME MEASURES: Presenting clinical features, therapy, and outcome. RESULTS: Four patients (female) between the ages of 56 and 75 years (mean, 65 years; median, 64 years) sought treatment for slowly evolving, painful, unilateral blue-gray swellings of the pretarsal tissues of the lower (1 case), upper (1 case), or both eyelids (2 cases). The swelling progressed to frank tissue necrosis and loss of full-thickness eyelid, with patchy sparing of the lid margin or lashes; in some cases, there was a very distinctive preservation of the pretarsal marginal artery across full-thickness eyelid defects. The lid loss characteristically involved the lateral one third of the lower eyelid (3 of 4 lids), the central part of the upper eyelid (3 of 4 lids) and, in one case, extending into the postseptal tissues in the inferotemporal quadrant of the orbit. In 3 patients, the pyoderma, often associated with a positive serum rheumatoid factor (three of three cases where measured), responded well to systemic immunosuppression and eyelid repair was undertaken during the quiescent phase. In a single patient, relapsing disease led to loss of the eye as a result of involvement of the globe and deep orbital tissues. CONCLUSIONS: Although an extremely rare condition, periocular pyoderma gangrenosum has a typical clinical appearance, and early recognition and immunosuppression will reduce the ocular morbidity.
Malignant pyoderma of the right ear developed in a 58-year-old woman, and it spread insidiously over the entire right side of the face. Pyoderma gangrenosum was diagnosed on the basis of clinical and histologic findings and the patient's history of ulcerative colitis and sclerosing cholangitis. After successive therapeutic failures with a score of conventional approaches over an 18-month period, oral cyclosporine, 10 mg/kg/day, was started. Significant healing of the skin was noted within 1 month. There was an associated remission of the patient's cholangitis and inflammatory bowel disease. The dosage of cyclosporine was progressively tapered and then discontinued after 7 months, when healing was complete. No relapse occurred in the subsequent 14 months, and there was an associated remission of the patient's cholangitis and inflammatory bowel disease. Cyclosporine merits serious attention for treatment of both pyoderma gangrenosum and sclerosing cholangitis.
The typical lesion of pyoderma gangrenosum is a rapidly extending necrotic ulceration, often associated with a system affection, inflammatory colitis, hematologic affections, rheumatic disorders. In about 30% of cases it develops on the site of a sometimes minimal injury. In the two cases reported one developed in the region of a graft after excision of a basal cell epithelioma, the other in a peritoneal drainage scar after colon resection. In patients with pyoderma gangrenosum, initial discussion must involve possible causes of the cutaneous necrosis, but in the cases reported the clinical appearance and rapid progression of the lesion, and negative results of serial bacteriologic and mycologic examinations were in favor of this diagnosis. An accurate diagnosis is essential since general corticotherapy is usually necessary.
Since 1954, 34 patients have attended St. Mark's Hospital with pyoderma gangrenosum in association either with ulcerative colitis (22 patients) or Crohn's disease (12 patients). Lesions were multiple in 71 per cent and over half were situated below the knees. Ulcerative colitis was active in 11 patients (50 per cent) and Crohn's disease was active in nine (75 per cent) when pyoderma gangrenosum was diagnosed. Associated illnesses--most commonly a seronegative arthritis affecting large joints--were present in 55 per cent and 92 per cent of cases respectively. A diffuse pustular rash appeared in six patients, synchronously with pyoderma in five. In a further seven patients (two with ulcerative colitis, five with Crohn's disease) the onset or course of pyoderma might have been linked to the presence of non-dermatological suppuration. Pyoderma resolved without intestinal resection in two-thirds of patients. When present at the time of surgical resection (15 procedures in 13 patients), pyoderma healed promptly in six cases, only with additional therapy in four cases and very slowly or not at all in five cases. Pyoderma gangrenosum occurs in both ulcerative colitis and Crohn's disease. Healing after intestinal resection is unpredictable both with respect to timing and extent of resection.
Pyoderma gangrenosum, cystic acne, and aseptic arthritis are clinically distinct disorders within the broad class of inflammatory diseases. Although this triad of symptoms is rarely observed in a single patient, a three-generation kindred with autosomal-dominant transmission of these three disorders has been reported as "PAPA syndrome" (MIM 604416). We report mapping of a disease locus for familial pyoderma gangrenosum-acne-arthritis to the long arm of chromosome 15 (maximum two-point LOD score, 5.83; recombination fraction [straight theta] 0 at locus D15S206). Under the assumption of complete penetrance, haplotype analysis of recombination events defined a disease interval of 10 cM, between D15S1023 and D15S979. Successful identification of a single disease locus for this syndrome suggests that these clinically distinct disorders may share a genetic etiology. These data further indicate the role of genes outside the major histocompatibility locus in inflammatory disease.
The authors summarize recent data on the etiopathogenesis and therapy of pyoderma gangrenosum and report on a 72-year-old patient for years suffering from recurring nodose lesions on his forelegs. The ulcerations first appeared a month before the patient's admission to author's Department. The course of the disease, patient's history and comprehensive clinical and laboratory findings pointed to the diagnosis of pyoderma gangrenosum. The patient was treated with systemic application of corticosteroids, and this therapy proved very effective.
Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative disease of undetermined cause. The diagnosis is based on clinical and pathologic features and requires exclusion of conditions that produce ulcerations. An atypical bullous variant (atypical pyoderma gangrenosum, APG) exists with clinical features similar to those of Sweet syndrome. Because PG is a rare disease, few large case series have been reported. Pyoderma gangrenosum was first recognized as a unique disease entity in the first half of the 20th century. Cumulative knowledge of PG is based on a handful of case series and multiple individual case reports. To augment that knowledge, we present our experience with a large number of patients over a significant time. We performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. The mean (+/- standard deviation) age of onset of PG and APG, respectively, was 44.6 +/- 19.7 years and 52.2 +/- 15.3 years. Lower extremity involvement was most common in PG, whereas upper extremity involvement was most common in APG. Associated relevant systemic diseases were seen in 50% of patients. Inflammatory bowel disease was the most common association in patients with PG, whereas hematologic disease or malignancy was most common in those with APG. Although a few patients were managed with local measures or nonimmunosuppressive treatment, the majority required oral corticosteroid therapy, often with systemic immunosuppressive treatment. PG patients required a mean 11.5 +/- 11.1 months of treatment to achieve remission compared with 9.0 +/- 13.7 months for patients with APG. Five patients (5.8%) had disease that was extremely refractory to multiple intensive therapies. The prognosis and disease associations for PG and APG appear to be different. Compared with PG, APG is more often associated with hematologic disease or malignancy, and remits more quickly.
We describe a case of pyoderma gangrenosum which presented with severe wound breakdown after elective hip replacement. The patient was treated successfully with minimal wound debridement and steroids. This diagnosis should always be considered when confronted with an enlarging painful skin lesion which does not grow organisms when cultured and fails to respond to antibiotic therapy, especially if there are similar lesions in other sites. In patients who have a past history of pyoderma gangrenosum, prophylactic steroids may be indicated at the time of surgery or may be required early in the postoperative period.
We report a retrospective study of 15 cases of pyoderma gangrenosum. The male/female ratio was 2, average age 40 years (range: 2-48 years). The typical ulcerous form was observed in 11 patients, whereas bullous pyodrma gangrenosum was noted in 2 patients and the granulomatous superficial form in 2 others. The leg was the most frequent localization. It was often associated with another skin localization. Two patients had neutrophilic pulmonary involvement which was concomitant to the skin ulcers. Association with internal disease was found in 8 patients. Histology showed vasculitis in 11 patients. In 9 of them, leukocytoclastic vasculitis was observed. Prednisone and clofazimine were the most frequently prescribed drugs. Th rate of recurrence of pyoderma gangrenosum was 46% independently of treatment. Occurrence of neutrophilic pneumopathy was rapidly fatal in two of our patients
Two cases of pyoderma gangrenosum are described that are atypical in the appearance of their skin lesions and their association with intestinal diseases.