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Pyoderma gangrenosum and sterile multifocal osteomyelitis preceding the appearance of Takayasu arteritis.

A 9-month-old infant had pyoderma gangrenosum (PG) and sterile osteomyelitis. Three years later the patient developed Takayasu arteritis (TA). Sterile osteomyelitis was reported in approximately 30 patients with different skin lesions, but never together with pyoderma gangrenosum. The association of PG and TA has been reported rarely, and then mainly in adults or in children over age 9 years. This rare association should alert physicians with a case of infantile PG of unknown etiology to consider the possible diagnosis of TA.

Dose-Response Relationship, Drug↗

Treatment of pyoderma gangrenosum with disodium cromoglycate.

The case of a 35-year-old black woman with a 10-year history of ulcerative colitis and a 3-year history of pyoderma gangrenosum is presented. The pyoderma gangrenosum was treated successfully with topical application of disodium cromoglycate.

Administration, Topical↗

[Pyoderma gangrenosum, ovarian carcinoma treated with Melphalan and acute myelomonocytic leukaemia: report of one case and literature review (author's transl)].

A 67-year-old woman suffered from an ovarian carcinoma with lymph nodes metastasis. During 3 years, she was treated with alkylating agents (Melphalan). At the end of therapy, no recurrence was observed. Two years later, she developed concomitantly pyoderma gangrenosum and acute myelomonocytic leukaemia. Death occurred rapidly. The association between pyoderma gangrenosum and acute leukaemia is discussed in the light of 16 cases previously reported in the literature. In this case, an induction of leukaemia by cytostatic drugs seems likely. The authors conclude that pyoderma gangrenosum may be considered as a cutaneous signs of acute leukaemia.

Aged↗

Does intestinal resection heal the pyoderma gangrenosum of inflammatory bowel disease?

A retrospective study of nine patients with active pyoderma gangrenosum at the time of operation for inflammatory bowel disease showed two patterns of postoperative skin healing: 1) prompt healing within 2 months, occurred in five patients with moderate to severe inflammatory bowel disease. 2) skin disease persisted in four others, healing only after a year. Three of these patients had mild ulcerative colitis, and in them, the operation was carried out in the hope of curing crippling pyoderma gangrenosum. The fourth patient had only an intestinal bypass for ileitis. Our observations suggest that prompt skin healing may occur after surgery in patients with severe inflammatory bowel disease, but not necessarily in those with milder bowel disease or in those in whom some bowel disease persists.

Adolescent↗

[A case of pyoderma gangrenosum involving the prostate gland after radiation therapy for prostate cancer].

A 76-year-old man complained of difficulty in urination and miction pain with abacterial pyuria after radiation therapy for prostate cancer. Transurethral resection of the prostate was performed and histopathologically widespread necrosis was observed in the prostate. Thereafter retention of urine and fever occurred and computed tomography scan revealed an abscess of the penile corpus. The abscess was drained, but the fever continued. He developed an abacterial lung abscess and abacterial necrotic ulcerating lesions on his back, his left leg and his lower abdomen. Macroscopic findings demonstrated typical features of pyoderma gangrenosum. Steroid treatment was initiated and the response to steroid therapy was dramatic. Finally urinary diversion using an ileal conduit was performed. We found few cases of pyoderma gangrenosum involving lesions other than those of the skin in the literature. This is the first report of pyoderma gangrenosum involving the prostate gland after radiation therapy for prostate cancer.

Aged↗

[Pyoderma gangrenosum and chronic myeloïd leukemia. Two new cases (author's transl)].

Two new cases of pyoderma gangrenosum with chronic myeloid leukemia are described and added to three others of the literature. Pyoderma gangrenosum is often bullous, painful and progresses quickly. It seems to have a bad prognosis in myeloid leukemia as in polycythemia vera, in which it appears lately as an evolution to myelofibrosis or acute leukemia transformation. Chemotactic disorders of polynuclear may be the main etiological factor in these cases.

Adult↗

Pyoderma gangrenosum first presenting as a recalcitrant ulcer of the ear lobe.

A 59-year-old Japanese man with pyoderma gangrenosum occurring at the unusual location of the ear lobe is herein reported. The patient was not associated with any other systemic diseases and had suffered from chilblains at the same site for ten years before the ulcer appeared. The ulcer followed the development of a purpuric exudative lesion and had neither an undermined nor a surpiginous border in the early lesion. It gradually increased in size after various conservative treatments, recurred within a month after being excised and became aggravated after the administration of potassium iodide. Repeated histopathology of the ulcer revealed a mixed inflammatory cell infiltrate with abscesses and an extravasation of red blood cells in the whole dermis, without showing leukocytoclastic vasculitis. A culture of the excised tissue yielded no growth. Laboratory tests were not specific and c-ANCA was also negative. The ulcer of the ear did dramatically respond to systemic predonisolone of 40 mg/day. The auricular and periauricular area are quite rare anatomical sites of this disease and the difference between pyoderma gangrenosum and cutaneous Wegener's granulomatosis is also discussed.

Ear Diseases↗

Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients.

Because the incidence of pyoderma gangrenosum (PG) is low, no prospective randomized controlled trials and only a few studies with case numbers of more than 15 patients have been published. To date no guidelines for treatment of PG have been established far. The aim of the study was to provide an evidence-based review of the literature and an evaluation of recommendations for PG treatment. We performed an electronic search using the PubMed database and the term "pyoderma-gangrenosum." Literature published in the English language during the past two decades was reviewed. All relevant studies that could be obtained regardless of the study design were evaluated for grades of recommendation and levels of evidence. Data on patient characteristics including severity of the disease, localization of lesions, associated diseases, and treatment procedures were abstracted and evaluated for therapeutic outcome. We conclude that therapeutic efficacy of systemic treatment with corticosteroids and cyclosporine is best documented in the literature for disseminated as well as for localized disease and should be considered first-line therapy. In cases that do not respond to this treatment, we recommend alternative therapeutic procedures (eg, systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; infliximab; or plasmapheresis), considering additional factors including associated diseases.

Evidence-Based Medicine↗

Successful therapy of refractory pyoderma gangrenosum and periorbital phlegmona with tacrolimus (FK506) in ulcerative colitis.

We describe two male patients with ulcerative colitis and refractory pyoderma gangrenosum including periorbital phlegmona in one case. Both patients were successfully managed with low dose oral tacrolimus (0.1 mg/kg bodyweight per day). Serum trough levels were closely monitored and maintained between 4 and 6 ng/mL. A rapid response was noted in both cases. Complete non-scarring skin restitution without side effects was accomplished in both cases. Low dose oral tacrolimus provides a valuable alternative treatment option for IBD patients with refractory pyoderma gangrenosum.

Adult↗

Pyoderma gangrenosum--response to topical nitrogen mustard.

We report a 69-year-old caucasian male patient with long-standing pyoderma gangrenosum; the lesions preceded the appearance of an IgA monoclonal gammopathy by 2 years. A number of systemic treatments, including high dose steroids and immunosuppressive agents, were poorly tolerated and resulted in serious side-effects. The skin and haematological conditions, however, were kept under control for 2 years with regular plasmapheresis. Pyoderma gangrenosum recurred as vascular access became exhausted but new lesions healed completely with topical application of 20% nitrogen mustard.

Administration, Topical↗

Pyoderma gangrenosum in a kindred. Precipitation by surgery or mild physical trauma.

Five cases of pyoderma gangrenosum occurring in a kindred are presented. Three of the cases occurred after abdominal surgery and tended to be confused with postoperative wound infections. Two cases occurred after superficial injury to the leg and were also thought to represent a peculiar form of cellulitis. None of the patients are known to have any of the underlying diseases usually associated with pyoderma gangrenosum. The cases are presented to alert the physician to this entity and to document the unusual familial occurrence.

Abdomen↗

Pyoderma gangrenosum occurring at multiple surgical incision sites.

Ten weeks after subtotal colectomy and ileostomy for ulcerative colitis, a 16-yr-old girl developed wound drainage and back pain. Massive ulceration and skin separation occurred at the abdominal wound incision as well as at the incision sites of a previous central venous line. A diagnosis of pyoderma gangrenosum was made. High-dose steroid treatment induced prompt healing of the abdominal wound as well as the catheter sites. Pyoderma gangrenosum has rarely been seen in surgical wounds. To our knowledge, this is the first reported case of pyoderma gangrenosum occurring simultaneously in multiple surgical incision sites in a patient with ulcerative colitis.

Adolescent↗

Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathy.

A 46-year-old man with a 3-year history of pyoderma gangrenosum was admitted with ulceration (6 x 5 cm), on the right leg. Previously he had been treated with tapering doses of prednisone (maximum dose 1 mg/kg per day); however, he had had a few exacerbations following each taper of prednisone dose. Immunoelectrophoresis demonstrated monoclonal IgA gammopathy of lambda light chains. Abdominal echography and abdominal computed tomographic scan revealed multiple splenic abscesses. Treatment was started with oral prednisone (1 mg/kg per day) and cyclosporin (5 mg/kg per day) and 6 weeks later complete remission was achieved. Systemic involvement in pyoderma gangrenosum is very rare, and according to our knowledge there are only a few cases with spleen involvement.

Cyclosporine↗

Pyoderma gangrenosum in two siblings: a familial predisposition.

We report pyoderma gangrenosum in two siblings with onset during childhood and no associated systemic abnormalities. The patients were born of nonconsanguineous, healthy parents. Treatment with oral corticosteroids produced an excellent clinical response, followed by recurrence after cessation of therapy. Steroids were restarted in combination with dapsone to prevent further recurrence.

Administration, Oral↗

Pyoderma gangrenosum in a renal transplant recipient: a case report.

Pyoderma gangrenosum (PG) is an ulcerative disease of the skin of unknown etiology. Its association with infection, autoimmune disease, inflammatory bowel disease, malignancy, and certain drugs suggests a hypersensitivity reaction. We herewith present a renal transplant recipient who developed PG. The patient presented with multiple necrotizing skin ulcers on both the upper and lower extremities associated with malaise, myalgia, arthralgia, weight loss and low-grade fever. To our knowledge, the association between PG and renal transplant has not been reported previously.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum after reduction mammaplasty in an otherwise healthy patient.

Pyoderma gangrenosum (PG) is a rare postoperative complication of plastic surgery of the breast. Initial signs and symptoms resemble those of infection, and antimicrobial therapy is usually initiated and fails before considering PG as a diagnosis. Therapy consists of immune modulators, and use of corticosteroids is frequent, as is local wound care. Sufficiently small wounds are allowed to heal secondarily, but larger wounds require coverage with either skin grafts or flaps. Long-term (1 year or more) postoperative surveillance is necessary because late failure of the graft or flap can occur.

Adult↗