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Vegetative pyoderma gangrenosum in chronic renal failure.

We report the case of a 58-year-old woman with painful recurrent erythematous lesions on both legs of 6 months' duration. The patient had been treated with haemodialysis for chronic renal failure for the previous 10 years. Physical examination revealed pea-sized erythematous nodules with sinus formation and discharge of bloody pus, which yielded neither bacterial nor fungal cells in culture. These lesions healed leaving cribriform scars, after bed rest, topical disinfection and systemic and in some areas intralesional corticosteroids. Cases of pyoderma gangrenosum are usually classified as one of four clinical variants: ulcerative, pustular, bullous and vegetative. Our case might correspond to a vegetative form in view of the development of sinuses, neutrophilic abscesses and palisading granuloma, even though no ulceration was observed.

Anti-Inflammatory Agents↗

Pyoderma gangrenosum. Abnormal monocyte function corrected in vitro with hydrocortisone.

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.

Adult↗

Behçet's disease-like presentation of bullous pyoderma gangrenosum associated with Crohn's disease.

A 47-year-old woman presented with a 2-month history of generalized arthralgia and a 10-day history of oral aphthous ulcers. After hospitalization, papulopustular lesions and perianal ulcerations developed. Pathergy test was positive and ophthalmological examination was normal. The presence of oral aphthous ulcers, genital ulcerations, papulopustular lesions and arthralgia, and the positive pathergy test suggested the diagnosis of Behçet's disease (BD). In a few days, positive pathergy reactions and papulopustular lesions evolved into bullous lesions, which were diagnosed dermatopathologically as pyoderma gangrenosum. Two days after the presentation of papulopustular lesions, the patient experienced diarrhoea accompanied by bloody stools and mucus. Histopathological examination of biopsy specimens showed no vasculitis but revealed findings suggestive of Crohn's disease. The patient responded well to treatment with systemic steroids and 5-aminosalicylic acid. Our case demonstrates that the differential diagnosis of BD and inflammatory bowel disease may be perplexing and that these two diseases may be closely related.

Behcet Syndrome↗

Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins.

Congenital deficiency of beta 2 integrin leucocyte adhesion molecules is a rare immunodeficiency and is often fatal. Neutrophils are unable to bind to ligands on the endothelium, and so cannot leave the circulation during inflammation or infection. When leucocyte adhesion deficiency (LAD) is caused by abnormally low expression of beta 2 integrins, it is termed LAD type 1. We describe a 5-year-old girl with a history of recurrent bacterial infections since early childhood who developed necrotic skin ulcers resembling pyoderma gangrenosum and a persistent circulating neutrophilia. Histologically, the lesions showed deep ulceration with a diffuse lymphohistiocytic infiltrate, but with a relative sparsity of neutrophils. Subsequent investigation revealed a complete absence of CD11a/CD18 beta 2 integrins on the surface of the patient's neutrophils, confirming the diagnosis of LAD type 1. The ulcers responded to treatment with oral prednisolone and colchicine.

CD11 Antigens↗

Pharmacological modulation of neutrophil phagocytic function in a patient with recurrent sepsis, pyoderma gangrenosum and impaired phagocytosis.

A 50-year-old man with recurrent life-threatening sepsis and a cutaneous condition resembling pyoderma gangrenosum, was found to have a defect of neutrophil phagocytic function. Phagocytosis could be enhanced by corticosteroids, both in vitro in a dose-dependent manner, and in vivo, when it was accompanied by rapid clinical improvement. Studies with steroid hormones and immuno-stimulatory drugs are described.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum and myeloproliferative disorders. Report of a case and review of the literature.

The exact mechanism involved in the pathogenesis of pyoderma gangrenosum (PG) still remains unclear, yet there is an increasing number of reports associating PG with immunologic abnormalities. A correlation between PG and myeloproliferative disorders has also been described. We describe a patient with chronic myelocytic leukemia in whom PG developed during the course of illness. We present an immunologic analysis of this case, speculation on the pathogenesis of PG, and a review of the literature. We report the futility of current therapeutic modalities in the treatment of PG.

Adult↗

Use of IGIV in the treatment of atopic dermatitis, urticaria, scleromyxedema, pyoderma gangrenosum, psoriasis, and pretibial myxedema.

There has been a rapid expansion in the use of IGIV for an ever-growing number of conditions. It is a product with an excellent safety record without the side effects of steroids or other immunosuppressive agents. There have been numerous recent advances in our understanding of the mechanisms of action of IGIV in many of the conditions for which it is being used, but there is still much to be learned. IGIV has had a major impact in neurology, haematology, immunology, rheumatology and dermatology. The limitations for IGIV are cost of the preparation itself and the logistical problems associated with its administration. Here we describe the published evidence for the use of high-dose IGIV in the dermatological conditions atopic dermatitis, urticaria, scleromyxedema, pyoderma gangrenosum, psoriasis and pretibial myxedema. These conditions have an emerging evidence base for hdIGIV which is relatively small consisting mainly of case reports and small case series. The outcomes in a number of these conditions appear encouraging, but as the reports are likely to reflect a bias for positive results, one must be cautious about drawing firm conclusions.

Dermatitis, Atopic↗

Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome) associated with hypogammaglobulinemia and elevated serum tumor necrosis factor-alpha levels.

Pyogenic aseptic arthritis, pyoderma gangrenosum, and cystic acne (PAPA) syndrome is an unusual triad that was recently mapped to a chromosome 15q mutation. We describe a patient from this kindred in whom hypogammaglobulinemia and elevated tumor necrosis factor-alpha serum levels were detected. The patient responded well to intravenous gammaglobulin and intra-articular corticosteroid therapy. Immune abnormalities can be found in PAPA syndrome and could be the consequence of the chromosomal abnormalities affecting candidate genes on this chromosome with subsequent abnormalities in cytokine or chemokine secretion. Rheumatologists should be alert for this syndrome. Correction of the immune abnormalities may be effective in controlling the disease manifestations.

Journal Article↗

[Pyoderma gangrenosum and vasculitis. Pathogenic discussion apropos of 3 cases].

Three cases and a pathogenic discussion. We report three cases of clinically typical pyoderma gangrenosum (PG) remarkable for the presence, at histology, of inflammatory vascular lesions of varying intensity. Case No. 1. A 15-year old boy without previous pathology was admitted for necrotic cutaneous lesions typical of PG on both legs. Biopsy in the peri-ulcerous rim showed cellular infiltration of the dermis, principally by granulocytes, associated with granulocytic vasculitis with fibrinoid necrosis (fig. 1 and 2). Laboratory examinations only showed a decrease of CH 50, and attempts at making an aetiological diagnosis of this case of PG met with failure. PG regressed under treatment with disulone combined with topical corticosteroid therapy. Case No. 2. A 62-year old woman with no previous severe disease was admitted for PG of her right leg at the ulcero-necrotic stage (fig. 3). Biopsy in the PG rim showed infiltration of the dermis, predominantly by granulocytes, associated with granulocytic vasculitis and fibrinoid necrosis of the walls of small vessels (fig. 4). Investigations in search of a cause revealed benign thyroid gland hyperplasia. PG was cured after a 5-week oral treatment with minocycline. Case No. 3. This 63-year old woman with arterial hypertension was admitted for PG of both legs. Some lesions at a very early stage were bullous (fig. 5). Biopsy in the peripheral swelling showed polymorphous cellular infiltration of the dermis with a predominance of granulocytes. These cells infiltrated the vessels the walls of which were turgid but without fibrinoid necrosis (fig. 6).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

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.

Anti-Inflammatory Agents↗

Pyoderma gangrenosum, vesicovaginal fistula and endometriosis. A case report.

A 47-year-old multiparous woman presented with endometriosis, true urinary incontinence, and the mucosal and skin lesions of pyoderma gangrenosum. The subsequent investigations, treatment and course are discussed, as well as the possible causal interrelationship between the above three conditions.

Endometriosis↗

Pyoderma gangrenosum complicating Felty's syndrome.

The case of a 54-year-old woman with Felty's syndrome whose course was complicated by mucocutaneous lesions clinically typical of pyoderma gangrenosum is described. Necrotizing sinusitis and saddle nose deformity were distinctive clinical features. Lymphocytic vasculitis and rheumatoid nodule formation observed within panniculus at the base of a cutaneous lesion and in a nasal mucosal lesion were unexpected histopathologic findings.

Felty Syndrome↗

Do brown recluse spider bites induce pyoderma gangrenosum?

Brown recluse spider bites are usually self-limited skin lesions that infrequently progress to bullae, ulceration, and scarring. We treated a patient with a documented brown recluse bite who had recurring lesions resembling pyoderma gangrenosum (PG) that persisted for months. Three other patients referred to Vanderbilt University because of probable brown recluse bite also had pyoderma for the first time after a suspected arthropod bite. The persistent and recurrent pyodermas in these four patients indicate that (1) brown recluse spider bites may not be self-limited but induce PG or PG-like lesions; (2) arthropod bites in general may induce PG in susceptible people; and (3) treatment of the PG-like lesions in these patients may be difficult.

Adult↗