Clinical University of Dermatology of Lisbon, Portugal.
Am J Clin Dermatol. 2011 Aug 1;12(4):271-6. doi: 10.2165/11586900-000000000-00000.
Acute generalized pustular psoriasis (AGPP) is a rare variant of psoriasis that can be lethal without proper treatment. It can be caused by the withdrawal of corticosteroids and, among its extra-cutaneous manifestations, liver abnormalities are frequently under-reported or attributed to drugs.
The aim of this study was to assess the clinical and laboratory data, treatment options, and disease outcome in patients with AGPP and to search for significant differences between subgroups of these patients.
This was a retrospective analysis of the clinical files from inpatients with AGPP observed in our department between 1973 and 2008. Statistical tests were performed at a significance level of 5%.
This was an inpatient, single-center study.
Outcome measures were a previous history of psoriasis, corticosteroid use before admittance, mortality rate, white blood cell count, absolute neutrophil count, and abnormalities in liver enzymes.
A total of 34 patients fulfilled the inclusion criteria, of whom 61% were men and 65% had a previous history of psoriasis vulgaris. Topical corticosteroids were applied by 50% of patients before admittance. Skin lesions remitted with methotrexate, etretinate, or acitretin treatment in all but two patients who died of sepsis. Abnormalities in liver enzymes were present in 47% of patients. Patients without a previous history of psoriasis had a significantly younger age at the first episode of AGPP. In the comparison between the groups of patients with and without liver abnormalities, a male preponderance and higher leukocyte counts were found in the former, with a positive correlation between the absolute neutrophil count and total bilirubin also being observed. Previous use of retinoids or methotrexate was not associated with these hepatic alterations.
Limitations of the data were that this was a single-center, retrospective study with a small sample size.
Withdrawal of systemic or topical corticosteroids can precipitate or worsen AGPP and these agents should not be used in these patients. Liver abnormalities can be considered an extra-cutaneous manifestation of AGPP. As in other series, no association between the use of drugs and changes in liver tests was found and therefore the deleterious withdrawal of efficient drugs, namely acitretin and methotrexate, should be avoided.
急性泛发性脓疱性银屑病（AGPP）是一种罕见的银屑病变体，如果治疗不当可能是致命的。它可能是由皮质类固醇的停药引起的，在其皮肤外表现中，肝脏异常经常被报道不足或归因于药物。
本研究旨在评估 AGPP 患者的临床和实验室数据、治疗选择和疾病结局，并寻找这些患者亚组之间的显著差异。
这是对 1973 年至 2008 年间我院住院患者的 AGPP 临床病历进行的回顾性分析。统计检验的显著性水平为 5%。
这是一项住院、单中心的研究。
结局指标为既往银屑病病史、入院前皮质类固醇使用、死亡率、白细胞计数、绝对中性粒细胞计数和肝酶异常。
共有 34 例患者符合纳入标准，其中 61%为男性，65%有寻常型银屑病病史。入院前，50%的患者曾外用皮质类固醇。除 2 例死于脓毒症的患者外，所有患者均用甲氨蝶呤、依曲替酯或阿维 A 治疗后皮肤病变消退。47%的患者存在肝酶异常。既往无银屑病病史的患者首次发生 AGPP 的年龄明显较小。在有肝异常和无肝异常两组患者的比较中，前者以男性为主，白细胞计数较高，且绝对中性粒细胞计数与总胆红素呈正相关。既往使用维 A 酸类或甲氨蝶呤与这些肝改变无关。
数据的局限性在于这是一项单中心、回顾性研究，样本量小。
全身或局部皮质类固醇的停药可诱发或加重 AGPP，因此不应在这些患者中使用这些药物。肝脏异常可被视为 AGPP 的皮肤外表现。与其他系列一样，未发现药物使用与肝试验改变之间存在关联，因此应避免停用有效的药物，即阿维 A 和甲氨蝶呤。
