| Objective:Analysis clinical characteristics of PG, investigate the methods how to deal with the disease, analysis the prognosis of the disease, in order to assist the diagnosis and treatment of the disease.Methods:Retrospective analysis clinical data of one case diagnosed with HED; retrieval reported cases of PG that published in the database of CNKIã€Pubmed and CBM at home and abroad from2000to2011, at the same time, analysis46cases which recorded more detailed information from53articles reported on PG in the past10years at home, and collect the general information such as sex, age, clinical manifestations, duration of disease, laboratory findings, treatment options and prognosis of the reported cases, and then analysis the etiology, pathogenesis, clinical manifestations, laboratory tests, histopathological examination and bone marrow cytology of PG in our country. Compared with foreign reports, we can analysis the risk population, the age at onset, the clinical manifestations, complications, treatment methods, prognosis of PG in our country.Result:The causes of PG are not clear. It has complex clinical manifestations, and can be associated with multi-system damage, the first diagnosis is often misdiagnosed, and prognoses are quite different. There is also no gold standard for the treatment of PG.(1)The case of PG with MDS we reported initiated with PG as the as the first symptom, refractory to glucocorticoid therapy, efficacy of imatinib treatment. At present, and failed to be followed up.(2) Review the literature of reported83cases of patients,49cases of mail,34cases of female, male and female incidence rate ratio about1.44.(3) Age:Minimum1year, maximum83years old, median age47years, the average age45years old. Patients were among on every age segment, while the peak Age was40-70(55.4%)(4)4patients were less than10years old, and none of them has combined system diseases. Eight cases aged from10to20years old, only2combined system diseases in2cases (25%). In every age segment over the age of20, over40%combined system diseases.(5) PG is a rare disease, and no data of incidence is founded at home. The incidence in the United States is about1/100000each year.30%of PG patients have a history of trauma and trauma. Review of the literature of81patients,11%of which have a history of trauma and trauma.(6) Forty percent of lesions only exist on lower extremity, but can be at any skin location. None of lesions only exist on upper extremity.(7) Laboratory tests:non-specific laboratory findings.34%have the elevated blood leukocytes. HGB of30%is lower than the normal.21cases (54%) with high ESR (>20mmH2O/h).11in24cases found bacteria in the culture.(8)38.5%of83cases were associated with an underlying systemic disease, most commonly ulcerative colitis, MDS.(9) Application of glucocorticoid in treatment patients with no reaction should consider whether the diagnosis is correct or complicated with MDS.(10) PG cases with lower RBC are suggested to have bone marrow examination to exclude MDS.(11)58patients,53patients with systemic glucocorticoid treatment. The total efficiency of glucocorticoid treatment is96.2%.(12) Cyclosporine is a first-line drug, and can be used in combination with glucocorticoids or alone. Conclusion:The etiology and pathogenesis of PG is not yet clear. Diagnosis can be difficult, and the biopsy specimen does not provide any pathognomonic information. The key in diagnosing PG is excluding other causes of cutaneous ulcers through biopsy, culture, and clinical acumen. About half of the patients are with related diseases, younger, with less chance of related diseases. The ratio is stable at40%in cases over the age of20. It is necessary to exclude the related diseases for cases over20. Systemic corticosteroids have generally been the most predictable, effective medication when delivered in adequate doses. Cyclosporine has proved to be a very helpful substitute therapy for patients whose PG is resistant to cortico steroid therapy or who have had serious side-effects. |
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