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Pyoderma gangrenosum complicating ulcerative colitis: successful treatment with methylprednisolone pulse therapy and dapsone.

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.

Adult↗

Pyoderma gangrenosum of the ocular adnexa: a rare condition with characteristic clinical appearances.

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.

Aged↗

Cyclosporine therapy for pyoderma gangrenosum associated with sclerosing cholangitis and ulcerative colitis.

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.

Administration, Oral↗

[Postoperative pyoderma gangrenosum. Apropos of 2 cases].

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.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum in inflammatory bowel disease.

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.

Adolescent↗

Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome maps to chromosome 15q.

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.

Acne Vulgaris↗

Pyoderma gangrenosum.

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.

Aged↗

[An unusual case of pyoderma gangrenosum with necrotizing granulomatous dermatitis].

Four distinctive clinical and histological variants of pyoderma gangrenosum have been proposed: (1) ulcerative, (2) pustular, (3) bullous, and (4) vegetative. The rate of progression and associated systemic diseases, as well as the histopathologic changes, can vary considerably between these clinical variants. A 64-year-old man presented with ulcerative pyoderma gangrenosum associated with renal cell carcinoma and IgA paraproteinemia; he responded to oral thalidomide. Histopathology revealed unusual findings with signs of leukocytoclastic vasculitis, basophilic degeneration of collagen and zones of suppuration with a palisaded histiocytic and epithelioid granulomatous infiltration throughout the dermis.

Dermatitis↗

Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions.

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.

Adolescent↗

Pyoderma gangrenosum. A diagnosis not to be missed.

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.

Aged↗

[Pyoderma gangrenosum. 15 cases].

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

Adolescent↗

Pyoderma gangrenosum after reduction mammoplasty.

The authors report a case of pyoderma gangrenosum in a 37-year-old woman that occurred at surgical sites after reduction mammoplasty. The diagnosis was delayed, but treatment with intralesional triamcinolone resulted in complete resolution o the condition. Pyoderma gangrenosum in this setting can mimic infectious causes of wound necrosis. Early recognition of its characteristics features may prevent unnecessary and ineffective treatment, thereby avoiding frustration for both patient and physician.

Adult↗

Post-colectomy peristomal pyoderma gangrenosum.

We report a patient with peristomal pyoderma gangrenosum which mimicked a necrotizing bacterial infection. It occurred as an extra-intestinal manifestation of chronic mucosal ulcerative colitis (MUC). Initially misdiagnosed as a cutaneous infection, it finally responded to intravenous high dose corticosteroid therapy.

Colectomy↗

Pyoderma gangrenosum of the penis.

Two cases of pyoderma gangrenosum involving the penis are presented. Treatment was difficult in both cases despite the use of high doses of prednisolone. In one case, the addition of thalidomide proved successful, whereas in the other, minocycline effected a cure.

Adult↗

Penile pyoderma gangrenosum.

We present a case of penile pyoderma gangrenosum (PG) that responded dramatically to an 8-week course of prednisone and has not recurred over a 6-month period. Although quite uncommon, penile PG should be a diagnostic consideration in any patient with non-healing ulcerative lesions of the penis. A correct diagnosis in this situation precludes unnecessary or harmful therapeutic interventions and leads to proper management.

Anti-Bacterial Agents↗

Topical disodium cromoglycate in the management of pyoderma gangrenosum.

A 52-year-old woman who had had ulcerative colitis for more than ten years experienced a third relapse of pyoderma gangrenosum of her lower extremities. She was treated with topical disodium cromoglycate. The dosage of salicylazosulfapyridine and steroids she was taking, and by which the ulcerative colitis, but not the pyoderma gangrenosum could be controlled, was not increased. Because pyoderma gangrenosum is often very difficult to treat and may require an aggressive approach using drugs that can cause serious side effects, we believe that a topical therapy is worth trying.

Administration, Topical↗

The management of leg ulcers in a patient with ulcerative colitis. This case study details the treatment of a patient with pyoderma gangrenosum on the shins of both legs.

Pyoderma gangrenosum is a rare condition which is sometimes associated with ulcerative colitis and Crohn's disease. The pathological basis is not completely understood but it may be caused by vasculitis. The lesions are typically said to have purple overhanging edges and a necrotic base. The condition may also occur with rheumatoid arthritis, multiple myeloma and leukaemia. The treatment of choice is systemic steroids but it may also respond to azathioprine, minocycline or clofazimine.

Adult↗