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Pyoderma gangrenosum in an elderly woman. Course marked by extensive morbidity.

A case of extensive medical and functional morbidity in an elderly patient during treatment of pyoderma gangrenosum is described. Pyoderma gangrenosum is an uncommon cause of ulcerative skin lesions often involving areas of skin trauma about the lower extremity. Because of the possibility of underlying disease and the special treatment requirements, the condition must be differentiated from other, more common causes of skin ulceration. Treatment includes local care, systemic corticosteroid therapy, and control of any underlying disease. Pyoderma gangrenosum serves as a model for the interactions of various types of morbidity in the healthcare of the elderly.

Administration, Oral↗

The use of high-dose immunoglobulin in the treatment of pyoderma gangrenosum.

BACKGROUND: Immunosuppressive medications such as corticosteroids and cyclosporin are the most commonly employed therapies in pyoderma gangrenosum. We describe a patient with multiple ulcers of pyoderma gangrenosum on the lower extremities in whom immunosuppressive therapy caused serious side effects and had to be discontinued but who was subsequently treated successfully with high dose intravenous immunoglobulin (IVIG). METHODS: IVIG was given intravenously at a dose of 400 mg/kg per day for 5 consecutive days. After 1 week there was an arrest in the progression of the ulcers and a marked reduction in pain. Two weeks later clinical improvement of the ulcers was observed. Subsequently, IVIG was given at a dose of 1 g/kg per day for 2 consecutive days. RESULTS: The treatment induced a dramatic clinical improvement of one ulcer and healing of the others. Side effects were minimal and well tolerated, and consisted of chills and a slight fever, which resolved with the administration of acetaminophen. CONCLUSION: We feel that IVIG can be used in patients with pyoderma gangrenosum in whom conventional therapies are ineffective or produce serious side effects.

Aged↗

Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: a rarely recognized extracutaneous manifestation of a neutrophilic dermatosis.

Neutrophilic dermatoses such as pyoderma gangrenosum are characterized by sterile, neutrophilic cutaneous infiltrates. Extracutaneous neutrophilic infiltrates can occur, primarily in the joints, lungs, heart, central nervous system, gastrointestinal tract, and eyes. Pulmonary disease is the most frequently reported extracutaneous manifestation of pyoderma gangrenosum and is characterized by patchy infiltrates or interstitial pneumonitis. We describe an adolescent with typical pyoderma gangrenosum who presented with cavitary pneumonia and responded completely to oral corticosteroids. In patients with inflammatory ulcers, extracutaneous neutrophilic disease should be considered, once an infectious process has been excluded.

Adolescent↗

[Pyoderma gangrenosum and biclonal gammapathy probably due to multiple myeloma (author's transl)].

The authors report the observation of pyoderma gangrenosum (P. G.) leading to the discovery of an underlying biclonal gammapathy, which despite the absence of bony lesions, almost certainly represents a malignant myeloma. The authors have reviewed the different known associations with pyoderma gangrenosum, with special reference to benign and malignant gammapathies. Rare cases of biclonal gammapathies with pyoderma gangrenosum have been published but none of a malignant nature. This therefore appears to be the first reported cases. The authors are aware of the reported tendency of pyoderma gangrenosum lesions to appear in areas of previous trauma, as may be the case in this patient. Finally systemic corticosteroid therapy produced a rapid remission of the skin lesions, but with the reactivation of known treated pulmonary tuberculosis.

Gangrene↗

Pyoderma gangrenosum associated with paroxysmal nocturnal haemoglobinuria.

A case of pyoderma gangrenosum of the lip occurring in association with paroxysmal nocturnal haemoglobinuria is described. This is an extremely rare association, which has been documented in the literature on only two previous occasions. Pyoderma gangrenosum (PG) is an uncommon ulcerative skin disorder of unknown aetiology. Its clinical appearance is often distinctive, with established lesions consisting of a necrotic ulcer surrounded by a ragged undermined violaceous edge. Lesions are usually painful and are most often found on the lower limbs but can occur on the trunk, head and neck. The diagnosis is essentially clinical as there are no characteristic histopathological changes. Since its original description in 1930, PG has been frequently associated with a number of underlying systemic diseases. Foremost among these are inflammatory bowel disease and inflammatory polyarthritis. The association with haematological disorders is also well recognized, and includes acute and chronic lymphocytic and myeloid leukaemias, polycythaemia rubra vera, myelofibrosis, myelodysplastic syndrome, essential thrombocythaemia, hypogammaglobinaemia, monoclonal gammopathy, multiple myeloma and non-Hodgkin's lymphoma. We report a case of PG occurring on the lower lip of a 26-year-old man recently diagnosed as having paroxysmal nocturnal haemoglobinuria (PNH).

Adult↗

Pyoderma gangrenosum after cesarean delivery.

In this reported case of pyoderma gangrenosum after cesarean delivery, deep ulceration involved the entire lower abdomen before immunosuppressive treatment was started. Initial high doses of prednisolone followed by low doses of cyclosporine A led to complete resolution of the skin lesions. This case report illustrates the importance of early diagnosis and effective systemic immunosuppressive therapy of pyoderma gangrenosum.

Abdomen↗

Treatment of pyoderma gangrenosum with cyclosporine: results in seven patients.

The mainstay of therapy for pyoderma gangrenosum has been corticosteroids, but many patients respond poorly. During the past 2 years we have treated seven patients who had pyoderma gangrenosum with cyclosporine after their condition proved resistant to conventional therapy. No evidence of permanent toxicity from cyclosporine was detected and treatment with other immunosuppressive agents was discontinued in five of seven cases. Tuberculosis was reactivated in one patient. Three patients had a remission, three had an intermediate response, and one did not respond. These results indicate that cyclosporine is useful in the treatment of patients with refractory pyoderma gangrenosum and suggest an immune mechanism in the pathogenesis of this disorder.

Adult↗

A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome.

OBJECTIVE: To describe a multigenerational family with transmission of an autosomal dominant disorder characterized by pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne. MATERIAL AND METHODS: We present a detailed case report of a 39-year-old man with arthritic changes in several joints, pyoderma gangrenosum, and cystic acne. Several relatives from three generations of his family underwent clinical and genetic investigations. The findings in this kindred are reported. RESULTS: Ten affected family members in three generations manifested variable expression of a pauciarticular, nonaxial, destructive, corticosteroid-responsive arthritis that began in childhood; pyoderma gangrenosum; and severe cystic acne in adolescence and beyond. Other less commonly associated features included adult-onset insulin-dependent diabetes mellitus, proteinuria, abscess formation at the site of parenteral injections, and cytopenias attributable to sulfonamide medications. Laboratory evaluation was nondiagnostic. Genetic studies excluded linkage to the major histocompatibility locus. CONCLUSION: The acronym of PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne) is suggested for this newly recognized pleiotropic autosomal dominant disorder. The nature of the genetic alteration in PAPA syndrome is unknown.

Acne Vulgaris↗

Pyoderma gangrenosum--a complication of chronic venous leg ulceration?

Three patients whose chronic venous leg ulcers were complicated by pyoderma gangrenosum are reported. Illnesses which may have predisposed to the development of pyoderma gangrenosum included Crohn's colitis, rheumatoid arthritis and small bowel perforation. Pyoderma gangrenosum should be suspected in susceptible patients whose venous leg ulcers undergo inexplicable and painful deterioration.

Aged↗

Intravenous cyclophosphamide pulses in the treatment of pyoderma gangrenosum associated with rheumatoid arthritis: report of 2 cases and review of the literature.

Pyoderma gangrenosum is a chronic ulceronecrotic inflammatory cutaneous disorder that can be associated with diseases such as rheumatoid arthritis (RA). No definitive treatment exists for this condition; steroids have been the mainstay of therapy, and the addition of immunosuppressives has been advocated. We describe 2 patients with pyoderma gangrenosum occurring in the setting of RA who, in addition to steroids, received pulse intravenous cyclophosphamide and had a remarkably good and lasting response. This is the first report of such a therapeutic approach. The pertinent literature is discussed. We conclude that pulse cyclophosphamide is another possible therapy for pyoderma gangrenosum.

Adult↗

Hyperbaric oxygen therapy for pyoderma gangrenosum.

Hyperbaric oxygen administered systemically has been advocated in the treatment of pyoderma gangrenosum. Owing to the serious potential risks of oxygen toxicity with systemic administration, we have devised a simplified technique for the administration of hyperbaric oxygen topically, and have used this method successfully to treat two patients with pyoderma gangrenosum, in whom the underlying etiology was not apparent, and specific therapy, therefore, not possible. The abundant formation of granulation tissue and the ability of the hyperbaric oxygen to arrest further extension of the ulceration suggests that this form of therapy might become the treatment of choice for all ulcers of the pyoderma gangrenosum type.

Aged↗

Pyoderma gangrenosum in acute myeloid leukaemia during immunosuppression.

We describe a patient who developed pyoderma gangrenosum during the remission phase of acute myeloid leukaemia whilst receiving maintenance therapy with methotrexate and 6-mercaptopurine. The spontaneous resolution of these skin lesions following discontinuation of chemotherapy suggests that these drugs may be of major significance in the aetiology of pyoderma gangrenosum. Nevertheless, 27 months later, a relapse of the leukaemia followed. Although pyoderma gangrenosum occurred during clinical remission, we cannot rule out a synergism of leukaemia and chemotherapy in its pathogenesis.

Adult↗

[Aseptic adenitis in pyoderma gangrenosum].

INTRODUCTION: Extra-cutaneous manifestations of neutrophilic dermatosis are rare. Symptomatic cases are usually multiform and confusing. We report a case with an aseptic lymph node abscess associated with pyoderma gangrenosum. CASE REPORT: A 28-year-old woman with past history of pyoderma gangrenosum was seen for abdominal symptoms and fever related to an epigastric mass. Due to signs indicating abscess formation, surgery was performed and led to total regression of the symptomatology. Recurrence was evidenced 3 months later with skin lesions which were negative on bacteriological examinations. The diagnosis of lymph node manifestations of pyoderma gangrenosum was retained. General corticotherapy was very effective. Relapse occurred again 6 years later and was treated with thalidomide. DISCUSSION: Lymph node involvement has been described in Sneddon-Wilkinson's disease associated with pyoderma gangrenosum. In neutrophilic dermatosis, pulmonary manifestations appear to be the least exceptional of the extra-cutaneous lesions. The pathophysiology has not been elucidated although recent hypotheses suggest that G-CSF could be involved. Corticotherapy is remarkably effective in most cases but thalidomide could be an interesting alternative treatment.

Abdominal Pain↗

Wall-eyed bilateral internuclear ophthalmoplegia (Webino syndrome) and myelopathy in pyoderma gangrenosum.

A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-ophthalmologic findings. Although it was initially attributed to lesions affecting the medial longitudinal fasciculus and the medial rectus subnuclei of the oculomotor complex in the midbrain the exact location of the lesion is still disputed. In the present case both myelopathy and Webino syndrome were probably due to vascular occlusive disease resulting from central nervous system vasculitis occurring in concomitance to pyoderma gangrenosum.

Adult↗

Pyoderma gangrenosum after coronary artery bypass grafting.

Pyoderma gangrenosum is a rare cutaneous disorder that may complicate recovery after a cardiac operation. The lesions appear to represent a necrotizing infection; however, they do not respond to surgical debridement and antibiotic therapy. The treatment is based on high-dose corticosteroids and concomitant treatment of any underlying systemic disease.

Aged↗

Successful treatment of pyoderma gangrenosum with topical 5-aminosalicylic acid.

Pyoderma gangrenosum in a twenty-nine-year-old woman with associated long-standing Crohn's disease was treated with topical 5-aminosalicylic acid cream. Significant improvement in the ulceration occurred despite progressive deterioration of the inflammatory bowel disease. The possible mode of action is reviewed. The efficacy, together with the lack of side effects, warrants further study into the therapeutic role of topical 5-aminosalicylic acid.

Administration, Cutaneous↗

Pyoderma gangrenosum: an unusual cause of periaural ulceration.

A case of pyoderma gangrenosum affecting the pinna and neck of a diabetic patient is reported. Appearances were suggestive of malignant otitis externa. A small biopsy resulted in rapid and aggressive exacerbation of the lesion. Pyoderma gangrenosum, although uncommon, should be considered as a cause of ulceration, particularly when the ulcer yields no growth on culture in a non-dependent area.

Diabetes Complications↗