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Pyoderma gangrenosum associated with erythroid hypoplasia.

Pyoderma gangrenosum is most commonly associated with inflammatory bowel disease and rheumatoid arthritis, but it has been associated with various haematological malignancies. We describe its association with primary erythroid hypoplasia without thymoma in an 80 year old woman who presented with septicaemia complicating urinary tract infection. Spontaneous healing of an extensive lesion was observed.

Aged↗

Deposits of complement and immunoglobulins in vessel walls in pyoderma gangrenosum.

Previous immunofluorescence studies on pyoderma gangrenosum (PG) proved negative. Biopsies from the ulcer edge of 8 patients with PG were examined by immunofluorescence microscopy. Deposits of complement C3 were seen in the vessel walls of all samples, IgM in three and IgA in one. Granular deposits of C3 were seen at the dermal--epidermal junction in 2 patients. Biopsies from clinically normal skin of 6 of the patients were negative. It is suggested that deposition of immune complexes in the dermal vessel walls may play a role in the pathogenesis of PG.

Blood Vessels↗

[Pyoderma gangrenosum (dermatitis ulcerosa)].

Even though pyoderma gangrenosum (dermatitis ulcerosa) is still considered to be a polyetiological syndrome with uncertain pathogenesis, immunological processes are attributed to it. It serves as an indicator of an underlying internal disease. A more than incidental occurrence is found of inflammatory intestinal diseases such as colitis ulcerosa, inflammatory joint disorders, among hematological diseases predominantly myeloic leukemia, paraproteinemia with and without plasmocytoma as well as inflammatory vascular processes. A variety of other simultaneous diseases have to be considered as isolated case reports; it remains for future investigations to decide if these will be classified among the merely coincidental diseases or as various manifestations of a common immunological process.

Anti-Bacterial Agents↗

[Pyoderma gangrenosum of the penis presenting as Fournier's gangrene: a case report].

We report a case of pyoderma gangrenosum of the penis presenting as Fournier's gangrene. A 77-year-old man who had undergone radiotherapy for localized prostate cancer 16 month earlier, presented with penile pain and fever. Symptoms began with erythema and induration on the dorsal surface of the penile shaft followed by spontaneous purulent drainage with severe pain. Magnetic resonance imaging was unremarkable except for swelling of the penile skin. Biopsy of the ulcerative penile lesion demonstrated a nonspecific inflammation without vasculitis or malignancy. Despite broad-spectrum antibiotics and debridement, the penile lesion extended and new satellite lesions developed as pustules on the glans. Since cultures were negative for aerobic and anaerobic bacteria, a course of intravenous prednisolone was then initiated at 100 mg/day. Within 24 hours the temperature normalized, progression of the penile lesions stopped and became convalescent. The steroid was then tapered and discontinued. The penile lesions healed slowly during the subsequent 1-month period. Based on the clinical course and histopathological findings as well as exclusion of other ulcerative conditions, a diagnosis of pyoderma gangrenosum was made. Penile involvement of this non-infectious ulcerating skin disease has rarely been reported. Pyoderma gangrenosum affecting the penile skin, such as that in present case, may show a similar presentation as Fournier's gangrene. Prompt differential diagnosis is mandatory since effective management for each processes is markedly different.

Aged↗

Minimizing the risk of post-operative pyoderma gangrenosum.

A 61-year-old woman with seropositive rheumatoid arthritis developed numerous ulcers due to pyoderma gangrenosum at suture entry/exit sites following an arthroplasty of the right hip when interrupted silk sutures were used to close the skin. When a subsequent arthroplasty was performed on the left hip and subcuticular Dexon sutures were used to close the skin only two small ulcers developed. Sixteen cases of pyoderma gangrenosum developing in surgical wounds have previously been reported. We recommend that surgery in patients with a history of pyoderma gangrenosum is performed when the pyoderma is clinically quiescent, and that subcuticular sutures are used for skin closure, thus avoiding puncturing the skin surface.

Adolescent↗

Two cases of pyoderma gangrenosum complicated with nasal septal perforation.

We report two patients with pyoderma gangrenosum complicated with nasal septal perforation. An 18-year-old woman and a 65-year-old man had typical lesions of pyoderma gangrenosum on the legs that responded well to oral prednisolone. Both patients complained of mild nasal discharge, and nasal fibroscopy revealed nasal septal perforation. Biopsy of the nasal lesions showed an active inflammatory infiltrate, mainly of neutrophils. Systemic investigations failed to show any pulmonary or renal lesions of Wegener's granulomatosis. Cytoplasmic immunofluorescent pattern antineutrophil cytoplasmic antibody was negative. In both cases, intense neutrophilic infiltration was observed not only in skin lesions but also in nasal lesions, which may indicate that the nasal lesions had a pathogenesis in common with the skin lesions.

Adolescent↗

Case of multiple myeloma associated with extramedullary cutaneous plasmacytoma and pyoderma gangrenosum.

A variety of cutaneous manifestations has been described in multiple myeloma including extramedullary cutaneous plasmacytomas, cutaneous amyloidosis, pyoderma gangrenosum, leukocytoclastic vasculitis, necrobiotic xanthogranuloma, scleromyxedema, Sweet's syndrome, subcorneal pustular dermatosis, scleredema, and plane xanthomas etc. An 89-year-old Korean man, who had been suffering from multiple myeloma 1 year previous, presented for evaluation of two nodules on the right side of the forehead, left side of the chest (7th rib area), and multiple ulcers with papulopustules on both the thigh and the left side of the chest (2nd rib area) during 15 days, which developed at the same time. A biopsy of a lesion which manifested as a cutaneous nodule on the right side of the forehead revealed dermal infiltration by well-differentiated plasma cells, similar to those found on a bone marrow biopsy, and a biopsy of the lesion manifested as a painful ulceration on the right thigh area showing dermal neutrophilic infiltration. Histologic findings were consistent with plasmacytoma and pyoderma gangrenosum, respectively. We present a case of multiple myeloma which developed extramedullary cutaneous plasmacytoma and pyoderma gangrenosum simultaneously, which is very rare. The patient was treated with a systemic steroid and conservative therapy.

Aged↗

Infliximab for treatment of pyoderma gangrenosum associated with clinically inactive Crohn's disease. A case report.

We report the case of a 57-year old female patient with refractory to treatment pyoderma gangrenosum associated with clinically inactive Crohn's disease. Pyoderma gangrenosum was successfully treated with Infliximab, a chimeric monoclonal antibody that inhibits tumour necrosis factor alpha (TNF-alpha). Our case report suggests that Infliximab, a therapeutic agent for refractory and fistulizing Crohn's disease, may also be safe and effective in the treatment of Crohn's disease associated pyoderma gangrenosum, even though the inflammatory bowel disease is clinically inactive and repeated infusions may be required for successful treatment.

Antibodies, Monoclonal↗

Pyoderma gangrenosum or leukaemia cutis?

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.

Antineoplastic Agents↗

[Pyoderma gangrenosum as a precursor of myeloid leukemia].

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.

Adult↗

Pyoderma gangrenosum in ulcerative colitis.

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.

Abdominal Muscles↗

Factitious panniculitis masquerading as pyoderma gangrenosum.

We report a case of factitious panniculitis masquerading as florid pyoderma gangrenosum in a 35-year-old woman. At presentation, she had tender, ecchymotic plaques over the lower trunk and limbs, and several biopsies showed active lobular panniculitis. However, the extensive ulceration that ensued was clinically persuasive for pyoderma gangrenosum. We elected to treat the inflammatory element symptomatically with a range of topical and systemic medications including clobetasol propionate, tacrolimus 0.1% ointment, prednisolone, dapsone, cyclosporin A and mycophenolate mofetil, none of which effected an improvement. The possibility of a factitious aetiology had been suspected from the outset, and when signs of clinical depression emerged, antidepressant therapy was initiated and the ulcers were encased in fibreglass casts. Within a short period, healing commenced and slowly progressed with scar formation. In retrospect, we consider the diagnosis to have been factitious panniculitis on the basis of strong circumstantial evidence and the disparity between the histological and clinical features.

Adult↗

[Pyoderma gangrenosum and paraneoplastic chronic polyarthritis disclosing Hodgkin's lymphoma].

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.

Arthritis↗

Pyoderma gangrenosum in a patient with bullous systemic lupus erythematosus.

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.

Cyclosporine↗

Culture and immunohistochemical evidence of Chlamydia pneumoniae infection in ulcerative pyoderma gangrenosum.

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.

Adult↗

Topical tacrolimus (FK506) in the treatment of recalcitrant parastomal pyoderma gangrenosum associated with Crohn's disease: report of two cases.

INTRODUCTION: Pyoderma gangrenosum is a rare idiopathic skin disorder associated with other diseases, including inflammatory bowel disease. The commonest site is the skin, but sometimes it can occur in the parastomal region. Most of these cases respond to treatment with systemic corticosteroids and cyclosporin or local Kenalog injections. METHODS: The following are two cases of parastomal pyoderma in patients not responding to the standard measures. These patients were treated with topical tacrolimus. RESULTS: These patients showed dramatic improvement in one week with complete resolution and re-epithelialization of skin within two weeks. CONCLUSION: Pyoderma gangrenosum is a difficult problem to manage and its early resolution is important. In these two reported cases, the improvement was dramatic, therefore topical tacrolimus should be considered early in the management.

Administration, Topical↗

Pyoderma gangrenosum associated with ulcerative colitis: treatment with disodium cromoglycate.

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.

Administration, Topical↗