Jadassohn-Pellizari型皮肤松弛症1例.pdf

DermatovenereolJun.2023,Vol.37,No.6Chin2023年6 月中国皮肤性病学杂志第37 卷第6 期Jadassohn-Pellizari型皮肤松弛症1例郭润，张晓红，王俊慧，徐晨琛，崔炳南摘商要患者女,2 8 岁，腹部及背部疝囊样丘疹、斑块8 年，加重3年余。皮肤科情况：皮损为大小不一卵圆形斑块，质软，中央可见凹陷，伴风团样斑块，触之有疝囊感。皮损组织病理示：角化过度，表皮萎缩，真皮浅层胶原轻度增粗红染，血管周围及胶原间少量淋巴组织细胞为主的浸润。弹力纤维染色可见真皮乳头层弹性纤维正常，真皮中层弹性组织消失。诊断：Jadassohn-Pellizari型皮肤松弛症。患者未予治疗。关键词斑状萎缩;Jadassohn-Pellizari型中图分类号R 758.6文献标志码B文章编号1001-7089(2023)06-0701-02DO110.13735/j.cjdv.1001-7089.202208084Anetoderma of Jadassohn-Pellizari:A Case ReportGUO Run,ZHANG Xiaohong,WANG Junhui,XU Chenchen,CUI Bingnan(Department of Dermatology,Guanganmen Hospital,China Academy of Chinese Medical Sciences,Beijing 100053,China)Abstract A 28-year-old female presented with oval hernioid papules and plaques on theabdomen and back for 8 years,which were aggravated for more than 3 years.Dermatological examination showed that the skin lesion was oval,soft,with atrophyand wrinkle on the surface,and with a feeling of hernia sac.Histopathology of thelesion showed that hyperkeratosis,atrophy of epidermis,slight thickening and redstaining of collagen in the superficial layer of dermis,and infiltration of a smallnumber of lymphoid tissue cells around blood vessels and between collagen.Elasticfiber staining showed that the elastic fiber of dermal papillary layer was normal,andthe elastic tissue of dermal middle layer disappeared.The diagnosis of anetoderma ofJadassohn-Pellizari was made.The patient was not treated.KeywordsMacular atrophy;Jadassohn-Pellizari1临床资料患者女，2 8 岁，腹部及背部疝囊样丘疹、斑块8年，加重3年余。患者8 年前无明显诱因先于脐上部出现丘疹,3年前皮损加重，逐渐累及后背部，并伴风团，自觉瘙痒。患者曾就诊于多家医院，考虑多发性脂囊瘤，未予治疗。患者既往体健,否认遗传疾病病史,家族成员中无类似疾病患者。体检：系统检查未见异常。皮肤科情况：腹部散在、背部密集多发大小不一卵圆形斑块，质软，表面萎缩，起皱，中央可见凹陷，伴风团样斑块，触之有疝囊感（图1）。皮损组织病理示：角化过度，表皮萎缩，真皮浅层胶原轻度增粗红染，血管周围及胶原间少量淋巴组织细胞为主的浸润（图2）。弹力纤维染色可见真皮乳头层弹性纤维正常，真皮中层弹性组织消失（图3）。诊断：Jadassohn-Pellizari型皮肤松弛症。患者未予治疗。作者单位中国中医科学院广安门医院皮肤科，北京10 0 0 53网络首发时间 2 0 2 3-0 3-1911:27【网络首发地址】https:/ 0 2 3年6 月第37 卷第6 期Chin J Derm Venereol,un.2023,Vol.37,No.6a图1a1c腹部、背部多发大小不一卵圆形疝囊样丘疹、斑块，表面萎缩起皱Fig.la lc Multiple oval hernioid papules and plaques in different sizes on the abdomen and back,with shrunk and wrinkle图2角化过度，表皮萎缩，真皮浅层胶原轻度增粗红染，血管周围及胶原间少量淋巴组织细胞为主的浸润(HE 100);图3弹力纤维染色可见真皮乳头层弹性纤维正常，真皮中层弹性组织消失(VG100)Fig.2Hyperkeratosis,atrophy of epidermis,slight thickening and red staining of collagen in the superficial layer of dermis,and infil-tration of a small number of lymphoid tissue cells around blood vessels and between collagen(HE 100);Fig.3 Elasticfiber staining showed that the elastic fiber of dermal papillary layer was normal,and the elastic tissue of dermal middle layerdisappeared(VG100)2讨论Jadassohn-Pellizari型皮肤松弛症属于原发性斑状萎缩的一种类型。斑状萎缩（macular atrophy）又称斑状皮肤松弛（anetoderma maculosa），通常起病于青年人，好发于胸部、背部、颈部和上肢。本病特征性皮损为局限性的松弛软垂皮肤，可呈疝囊样，伴皱纹萎缩，这是真皮弹力纤维局灶性缺失所致 ，但该病的发病原因尚不清楚。斑状萎缩一般可分为原发性和继发性两类。根据有无炎性皮损的区别，原发性斑状萎缩又包括有红斑风团先驱损害的Jadassohn-Pellizari型皮肤松弛症和无明显炎症性先驱症状的 Schweninger-Buzzi 型皮肤松弛症等。其中,Jadassohn-Pellizari型皮肤松弛症中,Jadassohn型前驱炎性损害多为红斑，而Pellizari型表现为先有风团样改变2 组织病理学主要改变为弹性组织局灶性或完全性缺失，尤其在真皮乳头层以及中层网状层，并常见断裂的弹性纤维残留。皮损早期有炎症反应时，可见血管及附属器周围有急性炎症细胞浸润，还可见浆细胞、组织细胞以及肉芽肿形成2 。Jadassohn-Pellizari型和Schweninger-Buzzi型皮肤松弛症在组织学上无明显差异3。根据组织病理学特点，本病应与真皮弹力纤维溶解症和皮肤松弛症等其他弹性组织的疾病相鉴别，前者主要为真皮中层组织全部消失，在真皮乳头层及网状层弹性保留有正常的弹性组织；后者真皮全层弹性纤维断裂或丢失，尤以真皮中层更为明显本例患者为青年女性，病程较长，无明确家族史，排除梅毒、麻风、结核、红斑狼疮、抗磷脂综合征等常见与继发性斑状萎缩相关的疾病45,同时结合皮损及组织病理结果，诊断为原发性斑状萎缩Jadassohn-Pellizari型皮肤松弛症。本病临床较为少见。早期炎症反应阶段,可试用青霉素治疗6，但（下转第7 0 6 页）http:/修回日期上接第7 0 2 页）.706中国皮肤性病学杂东2 0 2 3年6 月第37 卷第6 期Chin J Derm Venereol,2023.Vol.37.No.6脂及血糖。目前发疹性黄瘤病无特效治疗方法,部分患者皮疹可在血脂及血糖代谢异常控制后逐渐消退，对于少量无法消退的皮疹可以尝试采取激光、手术、化学剥脱等治疗。有报道使用超脉冲CO激光治疗黄瘤获得显著疗效11,同时患者满意度高、不良反应少、复发风险低。口服异维A酸治疗痤疮时应严密随访血脂情况。参考文献1 Kashif M,Kumar H,Khaja M.An unusual presentation oferuptive xanthoma:A case report and literature review J.Medicine（Ba l t i m o r e),2 0 16,95(37):e 48 6 6.2 Green WL,Benitez F,Velez L,et al.Man with a rash.Erup-tive xanthoma J.Ann Emerg Med,2013,61（5):595,603.3 Santos MA,Foulke G.Koebnerization and eruptive xantho-masJ.J Gen Intern Med,2019,34(9):1947-1948.4 Ladizinski B,Lee KC.Eruptive xanthomas in a patient withsevere hypertriglyceridemia and type 2 diabetes J.CMAJ,2013,185(18):1600.5 Solak B,Kara RO,Acikgoz SB,et al.First and only symp-tom of undiagnosed diabetes mellitus:eruptive xanthoma发展至萎缩期时无特效疗法2 。如有美观需求，可考虑局限性皮损行外科切除。本例皮损已发展至萎缩状态,未予特殊治疗，嘱可定期随访皮损变化。参考文献1】Bo l o g n i a JL,Jo r i z z o JL,R a p i n i R P.皮肤病学M.朱学骏，王宝玺，孙建方，等译.2 版.北京：北京大学医学出版社,2 0 17:16 6 6-16 6 9.2】赵辨.中国临床皮肤病学M.2版.南京：江苏凤凰科学技术出版社,2 0 17：12 41-12 43.3 Venencie PY,Winkelmann RK,Moore BA.Ultrastructural收稿日期2022-08-09J.BMJ Case Rep,2015,2015:bcr2015212160.6 Dicken CH,Connolly SM.Eruptive xanthomas associatedwith isotretinoin(13-cis-retinoic acid)J.Arch Dermatol,1980,116(8):951-952.7 Gollnick HP,Bettoli V,Lambert J,et al.A consensus-basedpractical and daily guide for the treatment of acne patientsJ.J Eur Acad Dermatol Venereol,2016,30(9):1480-1490.8 Leung AK,Barankin B,Lam JM,et al.Dermatology:how tomanage acne vulgaris J.Drugs Context,2021,10:2021-8-6.9 Oge LK,Broussard A,Marshall MD.Acne vulgaris:diag-nosis and treatment J.Am Fam Physician,2019,100(8):475-484.10 De Marchi MA,Maranhao RC,Brandizzi LI,et al.Effectsof isotretinoin on the metabolism of triglyceride-rich lipo-proteins and on the lipid profile in patients with acne J.Arch Dermatol Res,2006,297(9):403-408.11 Al-Niaimi F.Ultrapulsed CO,ablation in the treatment ofxanthelasma palpebrarum:high satisfaction treatment withlow recurrenceJ.J Dermatolog Treat,2022,33(2):1116-1118.收稿日期2022-04-24修回日期2022-07-16findings in the skinlesions of patients with anetoderma J.Acta Derm Venereol,1984,64(2):112-120.4 Mckee PH,Calonje E,Granter SR.皮肤病理学-一与临床的联系M.朱学骏，孙建方，译.3版.北京：北京大学医学出版社,2 0 0 6：10 45-10 47.5 Hodak E,David M.Primary anetoderma and antiphospholipidantibodies-review of the literature J.Clinic Rev AllergImmunol,2007,(32):162-166.6冯林，江阳，余音，等.Schweninger-Buzzi型斑状萎缩1例J.中国皮肤性病学杂志，2 0 19,33（5）：57 2-57 4.2023-02-15