1/3. Superficial granulomatous pyoderma: a case report of two Japanese patients and clinical comparison with foreign patients.Superficial granulomatous pyoderma (SGP) is an unusual variant type of pyoderma gangrenosum characterized by a benign course and specific histological features; it is quite a rare cutaneous disorder in japan. We reported two Japanese cases of SGP and compared the clinical features of 13 Japanese cases with those of 42 foreign cases. Case 1 was a 53-year-old female who presented with three indolent and ulcerative plaques with elevated edges on the back and the posterior portion of the left thigh. Case 2 was a 74-year-old female who presented with crusted and vegetative erythematous plaques on the left shoulder and left upper arm. Histological examinations revealed a characteristic three-layer granuloma in each case. Laboratory and physical examinations found no abnormalities. Topical corticosteroid, oral administration of minocycline, and/or additional supplementation with corticosteroid were effective. In comparison with foreign cases, Japanese cases show a possible differentiation in the ratio of sex and distribution of the ulcer. Females have a high incidence and the extremities are frequently involved area in Japanese cases. Up to now, 11 cases of SGP have been reported in japan. To our best knowledge, our patients are the first Japanese cases described in the English literature.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/3. The effect of infliximab on extraintestinal manifestations of Crohn's disease.OBJECTIVE: The purpose of this open pilot study was to assess prospectively the effect of infliximab on extraintestinal manifestations in patients with active Crohn's disease refractory to conventional treatment. patients AND methods: Twenty-two consecutive patients with Crohn's disease and one with ulcerative colitis presenting at least one of the known extraintestinal manifestations of Crohn's disease participated in the study. All the patients had Crohn's disease activity index (CAI) scores above 2. Each patient received an intravenous infusion of infliximab at a dosage of 5 mg/kg. A thorough questionnaire was used reviewing the extraintestinal manifestations of Crohn's disease such as erythema nodosum, pyoderma gangrenosum, eye lesions, arthritis or arthralgia, sacroiliitis or inflammatory back pain, hepatic disease, hematologic manifestations (megaloblastic, iron deficiency or hemolytic anemia, thrombocytosis), thrombosis, and nephrolithiasis. Musculoskeletal complaints were evaluated using the parameters intensity of pain, duration of morning stiffness (in minutes), presence of inflammatory back pain, Schober's test of the lumbar region, chest expansion, and distance from occiput to wall. The clinical assessment was performed on the day of the infusion and 2 weeks later. RESULTS: Eleven out of 23 patients had arthralgia of inflammatory nature, three others had evidence of active synovitis on physical examination, and 11 reported inflammatory back pain. Four patients suffered from protracted pyoderma gangrenosum; three had resistant aphthous stomatitis. Eleven patients had more than one extraintestinal manifestation. All four with pyoderma gangrenosum demonstrated significant improvement of their ulcers after one course of infliximab, with complete resolution of the skin lesions in three of them after repeated infusions of infliximab. Aphthous stomatitis completely responded in all patients after a single infusion. Seven out of 11 patients with arthralgia and seven out of 11 with inflammatory back pain/sacroiliitis experienced benefit after treatment with infliximab and reported at least partial clinical improvement in duration of morning stiffness, tender joint count, and visual analogue scale for pain. Only one of three patients with frank arthritis demonstrated clear improvement, and two others failed to respond to infliximab treatment. CONCLUSION: These preliminary results are encouraging and suggest a promising role of infliximab in the treatment of extraintestinal symptoms of Crohn's disease.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/3. Aberrant integrin (CR4; alpha(x)beta2; CD11c/CD18) oscillations on neutrophils in a mild form of pyoderma gangrenosum.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.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |