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Pyoderma gangrenosum occurring in a lower limb fasciocutaneous flap--a lesson to learn.

Pyoderma gangrenosum is a destructive cutaneous disease characterised by progressive painful ulceration. The occurrence of pyoderma gangrenosum at a surgical site is rare (especially if there is no predisposing illness), but is well recognised. We present a case of a 63-year-old man who developed erythematous ulcerative lesions due to pyoderma gangrenosum in and around a lower limb fasciocutaneous flap used to cover an exposed total knee prosthesis. The lesions were initially confused with postoperative wound infection. No predisposing disorder, other than the rarely reported association with osteoarthritis, was found. The diagnosis is important because its rapid detection not only avoids unnecessary treatment but also allows for prompt intervention with oral steroids. This case is presented to alert surgeons to the presence of pyoderma gangrenosum and its diagnostic confusion with postoperative wound infection.

Anti-Inflammatory Agents↗

Pyoderma gangrenosum with secondary pyarthrosis following propylthiouracil.

The association of pyoderma gangrenosum and arthritic symptoms is well documented. We present a rarely reported variant of this in a 44-year-old woman with pyoderma gangrenosum and bilateral large purulent effusions of her knees. She had no evidence of underlying rheumatoid arthritis or a specific seronegative spondyloarthropathy. Of note she had a history of Graves' disease for which she had been treated with propylthiouracil for 3 years and on investigation at this presentation had a markedly elevated perinuclear antineutrophil cytoplasm antibody (P-ANCA) level with specificities for IgM myeloperoxidase, IgG elastase and IgG lactoferrin. We believe this patient had pyoderma gangrenosum with secondary sterile pyarthrosis and a P-ANCA precipitated by propylthiouracil.

Adult↗

Pyoderma gangrenosum of the oral cavity, nose, and larynx.

Pyoderma gangrenosum is a clinical condition often associated with internal disorders and immunologic abnormalities. The diagnosis is deduced solely from clinical examination because no histologic or laboratory changes are pathognomonic. The lesions are quite painful and can be particularly disabling. Laryngeal involvement should elicit concerns for protection of the airway. This case demonstrated involvement of the oral cavity, nose, and larynx--as well as other skin sites of the body--with pyoderma gangrenosum. To our knowledge, this is the first reported case of intranasal and laryngeal pyoderma gangrenosum. Early treatment of any associated internal disorders and aggressive treatment with corticosteroids usually result in adequate control of this problem.

Biopsy↗

Pyoderma gangrenosum treated with hyperbaric oxygen therapy.

The management of pyoderma gangrenosum often requires systemic drug therapy, such as corticosteroids, sulfones, or immunosuppressants, either alone or in combination. Inconsistent response to therapy is a source of frustration to both patient and physician. Several reports in the literature document the successful treatment of pyoderma gangrenosum with hyperbaric oxygen therapy. In our patient, a woman with severe rheumatoid arthritis and diabetes mellitus, hyperbaric oxygen therapy not only promoted healing of pyoderma gangrenosum but permitted reduction of systemic corticosteroids.

Arthritis, Rheumatoid↗

Pyoderma gangrenosum and adrenocortical carcinoma.

Systemic disorders that have been associated with pyoderma gangrenosum include inflammatory bowel disease, rheumatoid arthritis, paraproteinemias, and hematologic malignancies. We report the case of a 55-year-old woman with pyoderma gangrenosum, IgA monoclonal gammopathy, and a cortisol-secreting adrenocortical carcinoma. Review of the literature revealed one previous case of pyoderma gangrenosum associated with a solid tumor; at autopsy, a carcinoid tumor and an adrenocortical adenoma were found. Our patient's rapid improvement after the carcinoma was resected and her subsequent disease-free course suggests that the two conditions were related. This case suggests that evaluation for underlying malignancy should be considered in patients with pyoderma gangrenosum.

Adrenal Cortex Neoplasms↗

Pyoderma gangrenosum in an HIV-infected patient.

Pyoderma gangrenosum has previously been reported in four patients with HIV infection. We describe here the case of a fifth HIV-infected patient with pyoderma gangrenosum and review the characteristics of the four previously reported cases.

Adult↗

Pyoderma gangrenosum: recognition and management.

Pyoderma gangrenosum, a progressive necrotizing and ulcerative disease of the skin, presents serious problems in diagnosis and management to physicians not familar with the entity. The clinical basis of the diagnosis is outlined, and the possible etiological mechanisms are reviewed. Treatment is primarily nonsurgical, relying on oral administration of corticosteroids and conservative local care of the ulcers.

Diagnosis, Differential↗

Pyoderma gangrenosum and related disorders.

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition with distinctive clinical characteristics and a frequent association with systemic diseases. Most frequently among the associated disease list are: inflammatory bowel disease, arthritis, and a variety of hematologic disorders. There is no specific treatment of PG; however, the effective treatment of an accompanying systemic disease, local therapy, and/or the use of topical or systemic agents known to be useful in neutrophil disorders, have been beneficial in most patients.

Humans↗

Serum C-reactive protein measurement in pyoderma gangrenosum.

A case of pyoderma gangrenosum is reported in a patient with a past history of ulcerative colitis. Serial C-reactive protein (CRP) concentrations were measured and were found to be markedly elevated at presentation. Levels were also raised during two subsequent relapses. CRP levels proved to be a useful objective indicator of disease activity and response to therapy, and reflected changes in disease activity before the erythrocyte sedimentation rate.

C-Reactive Protein↗

Pyoderma gangrenosum involving the foot. A case report.

Pyoderma gangrenosum is a rare and destructive inflammatory skin disease. The authors present a report of a patient with a classic case of pyoderma gangrenosum involving the foot. The diagnosis was made on the basis of clinical presentation and progression of the disease after differential diagnoses of common conditions were excluded. A brief overview of the disease process, its treatment, and its correlation with ulcerative colitis is provided.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum at the parastomal site in patients with Crohn's disease.

Pyoderma gangrenosum is a rare idiopathic skin condition often associated with other diseases, including Crohn's disease. We believe this is the first report of cases in which pyoderma gangrenosum was identified at the parastomal site in patients with Crohn's disease. Four episodes of the condition occurred in three patients. In these patients, pyoderma gangrenosum first appeared after two to three surgical procedures and between ten months and five years after the initial surgery. The disease course varied from resolution within two months with corticosteroid therapy to resolution only after revision or relocation of the stoma with resection of recurrent disease segments. Parastomal pyoderma gangrenosum at the ileostomy site in patients with Crohn's disease may be more common than previously thought. Optimal treatment has not yet been established, and treatment needs to be tailored for each patient.

Adult↗

Clofazimine in the treatment of pyoderma gangrenosum.

Ten patients with pyoderma gangrenosum, seven female and three male, 25 to 94 years old, mainly with multiple lesions, have been treated with clofazimine, 100 mg three times daily. Associated disease was registered in three patients: diabetes mellitus, a previous adenocarcinoma of the colon treated by hemicolectomy, and pustulosis palmoplantaris. In a further patient, M-component was found in the serum. In seven cases the lesions were completely healed by two to five months of therapy, and in three cases the ulcers healed partially. Side effects were redness of the skin in seven cases and dryness of the skin in two patients. No hematological side effects occurred. The working mechanism is still obscure.

Adult↗

[Pregnancy: a possible etiology of pyoderma gangrenosum. A case report and review of the literature].

OBJECTIVE: We report an unusual case of pyoderma gangrenosum at 26 weeks gestation and review other cases reported in the literature in order to examine the underlying causes and determine the appropriate diagnostic and therapeutic approach to this uncommon skin disease. Patient and methods. The patient developed an acute abdominal syndrome at 26 weeks gestation (laparotomy was non contributive) followed by pyoderma gangrenosum of the abdominal wall. Seven other cases have been reported in the literature associating pyoderma gangrenosum with pregnancy. We analyzed the clinical findings in these cases. RESULTS: Corticosteroid therapy successfully controlled pyoderma gangrenosum in our patient. Pregnancy outcome was favorable with little effect on the newborn. Complete healing was achieved after several months of treatment. CONCLUSION: Histological lesions are nonspecific. Diagnosis of pyoderma gangrenosum is generally based on clinical signs. Systemic disease is associated in half the cases (inflammatory bowel disease, myelodysplastic syndrome, rheumatoid arthritis). The impact on pregnancy appears to be limited. Treatment is based on high-dose systemic corticosteroid therapy or cyclosporin. The two drugs may have to be combined. Search for an associated disease and appropriate treatment is particularly important for the outcome of pregnancy.

Abdominal Wall↗

Immunologic and biochemical studies on a patient with pyoderma gangrenosum.

A patient with pyoderma gangrenosum without associated disease was studied. Routine investigations showed several abnormalities. High ESR, high alkaline phosphatase and glutamyl transferase (gamma-GT) levels, low iron and iron binding capacity, altered protein spectrum, presence of Staphylococcus aureus and group G hemolytic streptococci in ulcer culture, higher than normal antistreptolysin titers in the serum, and perivascular infiltration in the skin. Biochemical investigations aimed at finding any excessive hydrolytic activity did not reveal the presence of neutral proteases in circulation leaked out from PMN-leukocytes or elsewhere. Lysozyme levels were higher than normal, amylase and lipase levels were normal and 5' nucleotidase levels were below normal range. TCA-soluble polypeptides were present in the serum at levels two times higher than those in normal individuals. Immunochemical investigations showed the absence of immune complexes in the serum but presence of high amounts of C-reactive protein. Total complement activity was higher than normal and so was C3c level. Clq, C4, and C3d levels were within normal range. Biologic studies showed the presence of a factor in patient serum that made guinea pig skin hard, painful, erythematous, and eventually hairless, but not necrotic. A similar factor was either absent in normal serum or present in very low concentration. After salazopyrine treatment, all the above mentioned abnormalities corrected except that 5' nucleotidase activity remained slightly lower than normal, alkaline phosphatase levels remained slightly higher than normal, and C-reactive protein levels remained very high, though lower than those during intense disease activity.

Drug Combinations↗

Treatment of pyoderma gangrenosum.

Critical to the proper management of pyoderma gangrenosum are correct diagnosis, identification and treatment of any underlying disorder, and the proper choice of topical and systemic therapy. Many agents are available for the treatment of pyoderma gangrenosum. We review the current therapeutic options, their efficacy and side effects, and we offer some guidelines for their proper selection.

Administration, Cutaneous↗

Pyoderma gangrenosum and extensive caval thrombosis associated with the antiphospholipid syndrome--a case report.

Pyoderma gangrenosum is a rare cutaneous disorder frequently associated with systemic diseases. The authors report the case of a 28-year-old man with pyoderma gangrenosum and inferior cava vein syndrome due to thrombosis. The search for pyoderma gangrenosum-associated conditions (gastrointestinal, rheumatic, neoplasic, and infectious diseases) was negative. A prolonged partial thromboplastin time and a positive test for circulating anticardiolipin antibodies strongly suggested the diagnosis of the antiphospholipid syndrome. There are reports of at least three other cases of pyoderma gangrenosum associated with the antiphospholipid syndrome. This clinical case adds further evidence to the possible link between these disorders and warrants a search for anticardiolipin antibodies in patients with pyoderma gangrenosum.

Adult↗

Treatment of pyoderma gangrenosum with granulocyte and monocyte adsorption apheresis.

Pyoderma gangrenosum is an intractable skin disorder characterized by the development of erythematous pustules or nodules that rapidly progress to destructive, necrotizing, non-infective ulcers. We assessed the efficacy of granulocyte and monocyte adsorption apheresis (GCAP) therapy in two new patients, a 67-year-old man with ulcerations on his lower leg, and a 44-year-old man with turgid erythematous lesions with burrowing abscesses and sinus formation on his hip, groin, and thighs. Both patients received 10 GCAP treatments at 5-day intervals. Their skin lesions responded well. The 9 cmx6 cm ulcer on the lower right leg of the 67-year-old patient was completely covered by regenerated skin at the completion of therapy. The turgid skin lesions containing pustules and ulcers of the other patient showed amelioration and a marked decrease in the volume of exudate. Our results suggest that GCAP is a useful treatment modality for pyoderma gangrenosum.

Adsorption↗