Cases reported "Pyoderma Gangrenosum"

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1/6. Atypical hemorrhagic bullous pyoderma gangrenosum.

    A 55-year-old woman was seen in the Plastic and Reconstructive Surgery Clinic because of a nonhealing wound on her left leg of approximately 2 months' duration. She had an 8-month history of multiple wounds appearing on her arms and legs. The patient noted that the majority of the wounds had been treated and healed with the use of topical medications. One wound on her left leg had continued to enlarge rapidly despite topical treatments. Therefore, hyperbaric oxygen therapy had been administered for 15 sessions. No additional healing had occurred with this treatment. skin grafting was performed on the affected area. She developed ulcers and blistering lesions at surgical and nonsurgical sites after skin grafting. The patient was referred to the dermatology Department. Dermatologic examination revealed a deep, necrotic ulcer, 30 cm x 10 cm, with surrounding violaceous erythema on the donor area, ulceration (18 cm x 8 cm) on the graft area, a hemorrhagic bullous plaque (5 cm x 15 cm) over the right malleolus, scattered ecchymotic lesions and small hemorrhagic bullae on both legs, and small pustules around the staplers (Fig. 1a,b). Cutaneous biopsy of a new lesion revealed a focal, dense neutrophilic infiltrate, liquefaction degeneration in the center, lymphocytic and mild plasmacytic infiltration around the venules, and fibrinoid deposits in the walls and lumen of the vessels (Fig. 2a). In addition, excessive polymorphonuclear leukocytes and extravasated erythrocytes were present in the papillary and reticular dermis (Fig. 2b). The patient had a 14-year history of asthma bronchiale. physical examination did not reveal any abnormality, except for crackling rales at the base of each lung. Laboratory examinations were within normal limits, except for the sedimentation rate (55 mm/h). Protein electrophoresis, peripheral blood smear, abdominal ultrasound, and thorax and abdominopelvic computed tomography scans were all normal. Swab cultures from the ulcers were negative. Bullous pyoderma gangrenosum was diagnosed on clinical and histopathologic grounds. prednisolone 80 mg/day was started. Rapid epithelialization was observed within 2 months of treatment. The dose of prednisolone was gradually decreased to 20 mg/day, and was used as a maintenance dose for an additional 6 months. Complete improvement was achieved in 8 months. The patient has been followed up for approximately 1 year. There were no side effects observed during the treatment and in addition no new lesions developed at the follow-up.
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2/6. pyoderma gangrenosum-like ulcer in a patient with large granular lymphocytic leukemia.

    Large granular lymphocytic leukemia refers to a clonal expansion of lymphocytes that have abundant cytoplasm and azurophilic granules. The disease is characterized clinically by chronic neutropenia and it may be associated with recurrent pyogenic infections. Except for these infections, cutaneous manifestations of this disease have not been well characterized. We describe a patient with large granular lymphocytic leukemia, which was confirmed by molecular genetics studies, who had a pyoderma gangrenosum-like ulcer on his leg. Results of an evaluation of the histologic characteristics and the leukocytic immunophenotype of a skin biopsy specimen from the ulcer demonstrated large granular lymphocytes within the blood vessels. Cutaneous ulceration may be a manifestation of large granular lymphocytic leukemia, and this disease should be considered when diagnosing patients with otherwise unexplained pyoderma gangrenosum-like ulcers of the skin.
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3/6. A case of propylthiouracil-induced pyoderma gangrenosum associated with antineutrophil cytoplasmic antibody.

    A 27-year-old woman who had been receiving propylthiouracil for 2 years for Graves' disease presented with painful ulceration on the lower limbs which had first appeared 2 weeks previously. Well-circumscribed hemorrhagic ulcerations with ragged borders were noted on both legs. skin biopsy demonstrated a florid neutrophilic infiltrate and evidence of leukocytoclasis around small blood vessels in the papillary dermis compatible with the diagnosis of pyoderma gangrenosum. A highly positive perinuclear pattern of antineutrophil cytoplasmic antibody with specificities for IgM myeloperoxidase was observed. The authors think that propylthiouracil is associated with the occurrence of pyoderma gangrenosum in this patient.
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4/6. Pyoderma gangraenosum caused by rhizopus arrhizus.

    A rare case of pyoderma gangraenosum caused by rhizopus arrhizus is reported. The patient, a 50-year-old male farmer, was admitted to hospital complaining of gangrening and festering of the right upper arm with severe pain for nearly 2 months. A lesion was found on the inside of right upper arm. The central skin part of the lesion became black dry gangrenous, the periphery was deeply ulcerated with yellow-green pus. The necrotic crust, biceps, triceps and vessels inherited a histopathologically proven fungal infection. The fungus isolated was identified as rhizopus arrhizus. The infection was successfully treated with itraconazole.
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5/6. Pyoderma-gangraenosum-like ulcers associated with lupus anticoagulant.

    A 45-year-old women presented with a deep ulcerative lesion of the left breast. Two months later, a new cutaneous necrotic lesion located in the right pretibial areas was observed. The only serum abnormality was the presence of circulating lupus anticoagulant. Clinical features suggested the diagnosis of pyoderma gangraenosum and histologic examination showed microthrombosis in the dermal capillary vessels adjacent to a dense, mixed inflammatory infiltrate with some degree of vascular damage. Although thrombosis may be the result of vasculitis, it is likely that in our patient the presence of a lupus anticoagulant activity played a role in the pathogenesis of the cutaneous ulcers.
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6/6. Dermatological presentation of disease associated with antineutrophil cytoplasmic antibodies: a report of two contrasting cases and a review of the literature.

    Antineutrophil cytoplasmic antibody (ANCA)-associated disease (AAD) constitutes a pathological disease spectrum of a necrotizing vasculitis of small- and medium-sized vessels, extravascular granuloma formation, and necrotizing and crescentic glomerulonephritis, and also a clinical disease continuum which ranges from renal-limited disease to a widespread systemic vasculitis, including Wegener's granulomatosis and microscopic polyangiitis. In the latter, circulating ANCA are an aid to diagnosis and also may play a pathogenic part. Two contrasting patients with AAD are described, both of whom presented primary with dermatological features. These included a cutaneous purpuric vasculitis, orogenital ulceration, infarction of the fingertip, and pyoderma gangrenosum-like ulceration. These cases will familiarize dermatologists with both the concept and dermatological features of AAD.
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