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The Joint Commission on Accreditation of Healthcare Organizations recently approved new standards for pain assessment and management. These standards will influence the practice of dermatology nursing in every setting. Nurses play a critical role in the care of patients with pain. Pyoderma gangrenosum is used as the basis for applying the new pain standards.
A case of much less frequent association of the pyoderma gangrenosum and granulomatous disease of the colon (Crohn's disease of the colon) is presented. A literature review is made about the association of the same with other gastrointestinal affections. It is brought up to date the possible atiopathogenical mechanisms and therapeutical effects.
Aberrations of cellular immune functions in pyoderma gangrenosum (PG) may lead to nonspecific activation of inflammatory cells or to an imbalance of suppression leading to autoaggression (chronic ulceration). A patient with severe unremitting PG had anergy to a battery of seven skin test antigens. Mixed lymphocyte reactions, autologous mixed lymphocyte reactions, lymphocyte proliferative responses to antigens, and the production of leukocyte inhibitory factor were substantially suppressed, while the lymphocyte responses to mitogens were unaffected. Quantitative immunoglobulin and complement levels were normal. The inhibition of cellular immune functions was mediated by a factor in the patient's serum. This factor also inhibited lymphocyte functions of normal unrelated control subjects. Preliminary studies demonstrated that the factor is nondialyzable, heat stable, and not adsorbed by Staphylococcus A protein. Pulse therapy with large doses of corticosteroids resulted in dramatic clinical improvement.
In five patients with inflammatory bowel disease (three with ulcerative colitis, two with Crohn's disease), pyoderma gangrenosum developed on a lower extremity at the site of trauma. In these subjects, the pyoderma was not clearly correlated with disease activity.
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.
The spectrum of clinical presentation of haematological disease is wide. We highlight two features of this principle: a rare cause of a 'haematological' presentation and a possible haematological cause of a disease not normally considered as such. A case of systemic pyoderma gangrenosum presented with splenomegaly in the absence of a rash. A clonal gamma- and beta-T-cell receptor rearrangement was demonstrated. Such clones may be a general phenomenon involved in the pathogenesis of this condition.
We report a 52-year-old male Caucasian who presented with a peripheral erosive arthritis, then pyoderma gangrenosum, and six years later pancolitis and orogenital ulcers. The case illustrates an overlap that may exist between Behçet's syndrome and inflammatory bowel disease. In either condition seronegative erosive peripheral joint disease is an uncommon presenting feature.
Crohn's ileocolitis manifested in 2 Black patients with the exceptionally rare complications of pyoderma gangrenosum and sclerosing cholangitis. It is emphasized that Crohn's disease is very rare in the Black population of sub-Saharan Africa, and, as far as we are aware, no patient with large-bowel involvement has hitherto been reported.
We report a case of pyoderma gangrenosum (PG) mimicking a severe infectious skin disease in a woman with metastatic breast cancer. PG started at the site of an intramuscular injection administered a few days previously, and it subsequently extended. The skin disease was cured by high-dose corticosteroid therapy and clofazimine, but it marked a turn for the worst in the course of the breast cancer which became rapidly fatal.
We are presenting a 71-years old male patient with chronic skin lesions that appeared at multiple site of pacemaker implantations in the upper thorax. In spite of many treatment trials, no significant improvements were accomplished. Expanded dermatological tests including serial histopathological examinations were necessarily performed. Based on test results and clinical presentations, the diagnosis of pyoderma gangrenosum was made. Immediate steroid therapy allowed dramatic improvement of the chronic inflammatory condition. The patient is still undergoing a close dermatological and cardiological follow-up. Replacement of the pacemaker into the abdomen is currently being considered.
We describe the case of a 22-year-old black female with type 1 diabetes mellitus diagnosed when she was 12 years old. She first presented (March 1994) with pustules and ulcerations on the upper and lower limbs, trunk and scalp at the age 17. The diagnosis of pyoderma gangrenosum was made. Since presentation, changes in liver function were detected and subsequent study led to the diagnosis of sclerosing cholangitis. The diagnosis of ulcerative colitis was made after colonoscopy. Partial response was obtained with minocycline and clofazimine, but treatment with 5-aminosalicylic acid achieved no improvement of the ulcerations. Liver transplantation, followed by immunosuppressive therapy led to complete regression of the cutaneous lesions.
We have previously shown that the beta2 integrins CR3 and CR4 physically and functionally interact with urokinase receptors (uPAR) on neutrophil plasma membranes in an oscillatory fashion. In this study we have analyzed neutrophils from patient SC, a 34 y old African American female, with aberrant skin window results and recurrent perianal abscesses and pretibial lesions diagnosed as pyoderma gangrenosum. Although untreated migrating normal neutrophils exhibited 20 s sinusoidal oscillations in CR4-uPAR proximity, neutrophils from SC demonstrated a faster oscillation (10 s) in the form of a flyback sawtooth wave. This waveform mimicked that observed for normal neutrophils treated with subsaturating doses of the kinase inhibitors staurosporine, genistein, and erbstatin. As beta2 integrins are regulated by phosphorylation, we tested the hypothesis that the aberrant CR4-uPAR proximity oscillations seen in SC's neutrophils are due to defective kinase activity that might be balanced by a decrease in phosphatase activity. When SC's cells are exposed to subsaturating concentrations of the phosphatase inhibitor pervanadate, this caused the CR4-uPAR oscillations to become sinusoidal in shape with a 20 s period, as seen in normal migrating neutrophils. Although SC's neutrophils were deficient in spontaneous and N-formyl-methionyl-leucyl-phenylalanine-induced polarization, 0.5 microM pervanadate returned cell polarization to nearly normal levels, thus paralleling the acquisition of normal receptor interactions. Inasmuch as SC's cellular phenotype is mimicked by kinase inhibitors and corrected by phosphatase inhibitors, we suggest that a mutation(s) affecting the kinetics of intracellular signaling enzymes, but not blocking the pathway per se, may be responsible for this clinical state.
Behçet's syndrome (BS) is a rare multisystem disorder belonging to a group of neutrophilic dermatoses. We report a 65-year-old male patient who had suffered from recurrent painful orogenital ulcers for 50 years from the age of 15 and started developing pustular and bullous lesions evolving into non-healing ulcers similar to those seen in pyoderma gangrenosum (PG) two months prior to presenting to us. There was no evidence of systemic disease or malignancy. Routine baseline investigations were within normal limits. The patient was treated successfully with dapsone, antibiotics, and local wound care.
The case is presented of a patient with busulfan (Myleran) treated myeloid leukaemia, who developed bullous pyoderma gangrenosum. Skin symptoms appeared at the time when treatment was discontinued due to signs of bone marrow depression. The pyoderma disappeared following treatment with systemic steroid.
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.
We describe the case of a 18-year-old male patient who first presented with decompensated cirrhosis, fever and generalized lymphadenopathy. He had abnormal results for liver biochemical tests, with a hepatitic-like picture and high titre of antinuclear antibodies. According to the scoring system proposed by the International Autoimmune Hepatitis Group he had 'definite' autoimmune hepatitis and responded well to immunosuppressive treatment. One year later he developed pyoderma gangrenosum which was successfully treated with cyclosporine. Two years later he experienced bloody diarrhoea as a first presentation of ulcerative colitis. At that time both the cholestatic biochemical picture and the cholangiographic appearances of the biliary tree were consistent with primary sclerosing cholangitis. Despite the addition of azathioprine and ursodeoxycholic acid to his treatment regime he developed recurrent episodes of cholangitis and intractable pruritus for which he underwent successful liver transplantation.
A 48-year-old male with treated hypothyroidism underwent mechanical valve replacement for mitral valve regurgitation. After the operation, the patient developed progressive median chest wound ulceration. The wound did not heal with conventional therapies for mediastinitis such as administration of antibiotics, debridement of necrotic tissue or continuous irrigation. The entire surgical wound opened spontaneously. Bacterial cultures yielded negative and the wound biopsy specimen revealed non-specific inflammatory change. The anti-TSH receptor antigen level was high. Pyoderma gangrenosum based on auto-immune deficiency was diagnosed and high dose corticosteroid therapy was started. The wound healed completely in 5 months.
A 50-year-old man with a 7-year history of an ulcerative rash on the lower extremities had rapidly progressive renal failure. Monoclonal IgA (lambda) was detected in his serum. Kidney biopsy revealed proliferative glomerulonephritis with dominant IgA and C3 deposition, compatible with IgA nephropathy. Immunohistochemical staining with anti-lambda chain was negative and electron microscopic examination revealed deposits in subendothelial and mesangial areas. The findings on the skin biopsy were consistent with that of a pyoderma gangrenosum. The patient was treated with prednisolone pulse therapy followed by oral prednisolone. Renal function gradually and almost completely improved and proteinuria disappeared.