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自身炎症综合征的特点是无菌性炎症反复发作，无循环自身抗体或自身反应性T细胞。目前认为PASH综合征是一种遗传性自身炎症综合征，由多种中性粒细胞性皮肤病组成，包括坏疽性脓皮病（pyodermagangrenosum，PG）、痤疮和化脓性汗腺炎（hidradenitissuppurativa，HS）。上述三种症状同时出现实为罕见。
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Case
A36-year-oldmanpresentedwithabscessesincreasinginsizeonthebuttocksandaxillae.HismedicalhistoryincludedrecurrentPGonthelowerextremities,generalizedsevereacne,andleukocytoclasticvasculitis(LCV)thatdevelopedwhileonmgdailyprednisoneforalargePGlesion(Fig1).Heself-reportednohistoryofarthritis.Reviewofsystemswasotherwisenegative.Hewaspreviouslytreatedwithprednisone,dapsone,colchicine,mycophenolatemofetil,andcyclosporine.
患者男，36岁。因臀部和腋下脓肿不断增大入院。病史：双下肢复发性PG，全身严重痤疮，在口服强的松mg/d治疗PG时出现白细胞破碎性血管炎（leukocytoclasticvasculitis，LCV）（图1）。患者称无关节炎病史。全身检查也显示阴性。患者曾用药物：强的松，氨苯砜，秋水仙碱，霉酚酸酯和环孢菌素。
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Physicalexaminationrevealedcysts,papules,andpustuleswithhyperpigmentationandscarringonthebackandchest(Fig2).Multipleinflammatoryabscesseswithsinustractsandscarringwerepresentonthelowerbackandbuttocks(Fig3).A14-cmulcerwithviolaceous,underminedborderswaspresentonthelowerleg(Fig4).Scatteredpetechiaewithsurroundingpalpablepurpuraandhemorrhagicpatchesdevelopedextensivelyonthelowerextremities(Fig5,Fig6,Fig7).Thiseruptionpresentedonmultipleoccasionswhileonhigh-doseprednisonefortreatmentofPG,andresolvedwithoutdevelopingintoPG-likelesions.
体检发现囊肿，丘疹，脓疱，背部和胸部色素沉着和瘢痕形成（图2）。下背部和臀部出现多发性炎症性脓肿伴窦道和瘢痕（图3）。小腿上有一个溃疡，大小为14cm，紫色，边界被破坏（图4）。双下肢有分散的瘀点，周围有可触知的紫癜和出血性斑块（图）。用药高剂量的强的松治疗PG时，出现了上述症状，恢复后未进展成其他PG样病变。
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LaboratoryevaluationhadpreviouslyshownabimodalIgGandIgAmonoclonalgammopathy.Otherserologiesthatwerenegativeornormalincludedantinuclearantibodies;hepatitisA,B,andC;rheumatoidfactor;HLA-B27;lactatedehydrogenase;peripheralsmear;cryoglobulin;cryofibrinogen;andCD4:CD8ratio.Genetictestingoftheproline-serine-threoninephosphatase-interactingprotein1(PSTPIP1)generevealednomutations.Askinbiopsywastakenofthepalpablepurpuraontheleftlateralthigh;histochemicalstainingrevealedaperivascular,interstitialinfiltrate治白癜风的医院哪家好白癜风软膏能不能治白癜风