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Pyoderma gangrenosum in association with juvenile rheumatoid arthritis.

A 17-year-old girl presented with multiple, painful, erythematous blisters and ulcerated lesions on the shins and buttocks. She also had arthralgia. She had suffered from juvenile rheumatoid arthritis (JRA) and received anti-inflammatory agents and oral glucocorticoids for eight years. A biopsy of a lesion showed epidermal ulceration with marked neutrophilic infiltrates in the dermis. The patient was diagnosed with pyoderma gangrenosum (PG). PG is an uncommon cutaneous ulceration within the spectrum of the neutrophilic dermatoses that is reported in association with a number of systemic disorders, including inflammatory bowel disease, hematologic disease, internal malignancies, arthritis, immune abnormalities, and solid tumors. To our knowledge, this is the first reported case of PG associated with JRA.

Adolescent↗

Splenic infarction and tissue accumulation of crystals associated with the use of clofazimine (Lamprene; B663) in the treatment of pyoderma gangrenosum.

A male patient 68 years, suffering from pyoderma gangrenosum which was resistant to conventional treatment, received clofazimine 400 mg daily for 5 months, then reducing to 300 mg daily for the next 6 months. Eleven months after starting the drug, he was admitted to hospital with severe abdominal pain, laparotomy revealing infarction of the spleen, with violaceous congestion of the small bowel. The spleen was removed and post-operative recovery was satisfactory. Histopathological examination of the spleen (removed at operation) and of tissue from a duodenal biopsy (taken postoperatively) showed large numbers of striations and outlines suggestive of crystal deposition. Mesenteric lymph node revealed a massive accumulation of crystals in cortical and medullary sinuses. The findings emphasize that clofazamine should not be used in high dosage over prolonged periods of time, except under close clinical and laboratory supervision, and for conditions not amenable to other drugs.

Aged↗

The treatment of pyoderma gangrenosum with sodium cromoglycate.

The successful treatment of patients with pyoderma gangrenosum by means of the local application of sodium cromoglycate solution is reported. One of the patients also had chronic active hepatitis and a background history of ulcerative colitis. Possible mechanisms underlying the apparent success of this form of treatment are discussed.

Administration, Topical↗

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↗

Malignant pyoderma.

Malignant pyoderma is a rare, chronic, destructive ulcerating skin disease of unknown cause that affects young adults, usually males. In the seven previously reported cases, the lesions were distributed mainly about the head and neck region, and some were associated with neurological disturbances. The disease is progressive but responds to high doses of systemically administered steroids. We here describe an additional case and a follow-up of a previously reported case that was managed successfully with corticosteroid and cyclophosphamide therapy.

Adolescent↗

Bullous pyoderma gangrenosum and multiple myeloma.

The previously unrecorded association of superficial bullous pyoderma and IgG-producing multiple myeloma is described. A rapid response of the cutaneous manifestations was achieved by treatment of the malignant paraproteinaemia.

Antibodies, Neoplasm↗

Pyoderma gangrenosum treated with cyclosporin A.

A 49-year-old woman with a 14-year history of pyoderma gangrenosum unresponsive to a variety of systemic and topical treatments was given oral cyclosporin A (CyA), a potent immunosuppressive agent widely used in organ transplantation for the prophylaxis of acute graft-versus-host disease. Improvement and subsequent healing of the lesions was evident within 3 weeks of starting treatment and has continued for the duration of treatment (12 months).

Adult↗

Development of folliculitis and pyoderma gangrenosum in association with abdominal pain in a patient following treatment with isotretinoin.

A 19-year-old man with severe acne developed extensive scalp folliculitis and later superficial pyoderma gangrenosum following treatment with isotretinoin. A cyclical neutropenia was noted and bone marrow findings suggested myelodysplasia. We believe that isotretinoin was implicated in the development of overt symptoms in this patient whose haematological condition was previously asymptomatic.

Abdominal Pain↗

Leukocytapheresis treatment for pyoderma gangrenosum.

A 42-year-old man presented with painful erythema with pustules and multiple small ulcers on the shins. He had suffered from ulcerative colitis (UC) and received oral glucocorticosteroid and salicylazosulfapyridine therapies for 7 years. Biopsy of the lesion demonstrated mixed cellular infiltrates with dominant neutrophils. The patient was diagnosed with pyoderma gangrenosum (PG) and underwent leukocytapheresis (LCAP), an extracorporeal leucocyte removal therapy, once a week for 5 weeks without changing the doses of the oral medications. The skin lesions as well as clinical signs of UC rapidly improved after LCAP, and no recurrence was seen during a follow-up period. There were no major complications during LCAP. LCAP will provide an effective and safe tool for the treatment of PG.

Adult↗

Systemic pyoderma gangrenosum responding to infliximab and adalimumab.

Pyoderma gangrenosum (PG) is frequently associated with constitutional symptoms as part of a nonspecific inflammatory response. However, extracutaneous discrete aseptic neutrophilic infiltrates are extremely rare. We report a patient with idiopathic PG with splenic and psoas muscle involvement. His disease was extremely aggressive and was unresponsive to conventional immunosuppressive therapy. His cutaneous and extracutaneous PG cleared with infliximab and adalimumab.

Adalimumab↗

Pyoderma faciale in a patient with Crohn's disease.

We wish to report the progress of a patient with pyoderma faciale and Crohn's disease. The patient is interesting in that on two occasions the relapse in her skin condition coincided with the introduction of non-steroidal anti-inflammatory drugs. Therapy with isotretinoin was effective and well tolerated.

Adult↗

Recalcitrant pyoderma gangrenosum--two cases successfully treated with cyclosporin A.

The successful use of cyclosporin A (CSA) in organ transplantation is now well established. In recent years its usefulness has extended to the treatment of cutaneous autoimmune disorders, including pyoderma gangrenosum (P.G.). We report two further cases of recalcitrant P.G., both associated with rheumatoid arthritis (R.A.) which responded to low dose CSA.

Aged↗

Pyoderma gangrenosum complicating hysterectomy for fibroids.

Pyoderma gangrenosum is a destructive, non-infective ulceration of the skin. The case presented illustrates a rare but important presentation in a patient following a total abdominal hysterectomy and bilaterial salpingo-oophorectomy (TAH and BSO).

Female↗

Pyoderma gangrenosum associated with Takayasu's arteritis.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by destructive, necrotizing and noninfective ulceration of the skin mostly on lower extremities. PG is well known as a complication of Takayasu's arteritis in Japan. However, this association is not commonly observed in North American and European patients. We describe a case of PG that was associated with Takayasu's arteritis who was successfully treated with systemic cyclosporin. We have reviewed 35 well-documented PG cases with Takayasu's arteritis in comparison to 106 PG cases without Takayasu's arteritis. The results demonstrate that this association occurs predominantly in young females and that these cases exhibit more widespread PG lesions.

Adult↗

Pyoderma gangrenosum associated with hidradenitis suppurativa.

Pyoderma gangrenosum (PG) is associated with a number of systemic diseases. PG in association with hidradenitis suppurativa (HS) has been rarely reported. We describe six patients (three men, three women; aged 35--51 years), who developed PG on a background of HS. The onset of PG occurred only after HS had been present for at least two decades. No relationship in disease activity between the two conditions was observed. Three patients described previous severe adolescent acne vulgaris, one had concurrent systemic lupus erythematosus and another had chronic iron-deficiency anaemia. The course of PG was severe and refractory in four patients, who required treatment including high-dose oral corticosteroids, ciclosporin, intravenous immunoglobulin and intravenous cyclophosphamide.

Adrenal Cortex Hormones↗