{"paper_id":"23e5d4cc-dfa1-4aa2-9127-82bb7c8be57c","body_text":"Primary Cutaneous Endometriosis of Umbilicus\nVol. 29, No. 5, 2017 621\nReceived September 20, 2016, Revised October 19, 2016, Accepted for \npublication November 16, 2016\nCorresponding author: Bark-Lynn Lew, Department of Dermatology, Kyung \nHee University Hospital at Gangdong, 892 Dongnam-ro, Gangdong-gu, \nSeoul 05278, Korea. Tel: 82-2-440-7329, Fax: 82-2-440-7336, E-mail: \nbellotte@hanmail.net \nThis is an Open Access article distributed under the terms of the Creative \nCommons Attribution Non-Commercial License (http://creativecommons.\norg/licenses/by-nc/4.0) which permits unrestricted non-commercial use, \ndistribution, and reproduction in any medium, provided the original work \nis properly cited.\nCopyright © The Korean Dermatological Association and The Korean \nSociety for Investigative Dermatology\npISSN 1013-9087ㆍeISSN 2005-3894\nAnn Dermatol Vol. 29, No. 5, 2017 https://doi.org/10.5021/ad.2017.29.5.621\nCASE REPORT\nPrimary Cutaneous Endometriosis of Umbilicus\nSeung-Hee Loh, Bark-Lynn Lew, Woo-Young Sim\nDepartment of Dermatology, Kyung Hee University College of Medicine, Seoul, Korea\nCutaneous endometriosis is defined by the presence of endo-\nmetrial glands and/or stroma in skin and represents less than \n1% of all ectopic endometrium. Cutaneous endometriosis is \nclassified as primary and secondary. Primary cutaneous en-\ndometriosis appears without a prior surgical history and sec-\nondary cutaneous endometriosis mostly occurs at surgical \nscar tissue after abdominal operations. The most widely ac-\ncepted pathogenesis of secondary endometriosis is the iatro-\ngenic implantation of endometrial cells after surgery, such as \nlaparoscopic procedures. However, the pathogenesis of pri-\nmary endometriosis is still unknown. Umbilical endome-\ntriosis is composed only 0.4% to 4.0% of all endometriosis, \nhowever, umbilicus is the most common site of primary cuta-\nneous endometriosis. A 38-year-old women presented with \nsolitary 2.5×2.0-cm-sized purple to brown colored painful \nnodule on the umbilicus since 2 years ago. The patient had \nno history of surgical procedures. The skin lesion became \nswollen with spontaneous blee ding during menstruation. \nThe skin lesion was diagnosed as a keloid at private hospital \nand has been treated with lesional injection of steroid for sev-\neral times but there was no improvement. Imaging studies \nshowed an enhancing umbilical mass without connection to \ninternal organs. Biopsy specimen showed the several dilated \nglandular structures in dermis. They were surrounded by en-\ndometrial-type stroma and perivascular infiltration of \nlymphocytes. The patient was diagnosed as primary cuta-\nneous endometriosis and skin lesion was removed by com-\nplete wide excision without recurrence. We report an inter-\nesting and rare case of primary umbilical endometriosis mis-\ntaken for a keloid and review the literatures. (Ann Dermatol \n29(5) 621∼625, 2017)\n-Keywords-\nCutaneous endometriosis, E ndometriosis of umbilicus, \nPrimary cutaneous endometriosis, Umbilical endometriosis\nINTRODUCTION\nEndometriosis is histopathologically defined by the pres-\nence of endometrial glands and/or stroma outside of the \nendometrium1. Ectopic endometriosis could be developed \nin many other tissues, most commonly affects pelvic or-\ngans such as ovaries, fallopian tubes, uterine ligaments, \npelvic wall2. Primary cutaneous umbilical endometriosis, \nwhich is also known as Villar ’s nodule, is a rare manifes-\ntation of endometriosis 3. Secondary endometriosis mostly \noccurs at surgical scar tissue after abdominal operations 4. \nThe most widely accepted path ogenesis of secondary en-\ndometriosis is the iatrogenic implantation of endometrial \ncells after surgery, commonly after laparoscopic proce-\ndures\n5. However, the pathogenesis of primary endome-\ntriosis is still unknown.\nTo date, umbilical endometriosis has been reported to rep-\nresent about 0.4% to 4.0% of all endometriosis and ac-\ncounts for 30% to 40% cases of cutaneous endometriosis. \nAmong cutaneous endometriosis, primary umbilical endo-\nmetriosis was considered even less common.\nCASE REPORT\nA 38-year-old multigravida fema le visited our department \nbecause of a painful nodule on  her umbilicus. The patient \nrecalled that the lesion was observed 2 years ago and the \n\nSH Loh, et al\n622 Ann Dermatol\nFig. 2. (A, B) Specimen showed le-\nsions in the superficial dermis and \ndeep dermis comprising dilated glan-\ndular structures, surrounded by cel-\nlular endometrial-type stroma (H&E; \nA: ×40, B: ×100, respectively).\nFig. 1. About 2.5×2.0-cm-sized brownish to purple colored \nnodule on the umbilicus.\nlesion became swollen with spontaneous frank bleeding \nduring menstruation. The pati ent had no history of surgi-\ncal procedure, nor any family  history of malignancy. The \nnodule was first diagnosed as a keloid at a private clinic \nand had been treated with intralesional injection of steroid \nfor several times without any signs of improvement. \nPhysical examination revealed a 2.5×2.0-cm-sized brown-\nish to purple colored nodule on the umbilicus (Fig. 1). \nImaging studies were carried out for differential diagnosis \nwith Sister Mary Joseph nodu le and keloid. Umbilical ul-\ntrasonography showed a mass with heterogenous echoge-\nnecity, increased vascularity and abdominal computed to-\nmography (CT) revealed enhancing mass at umbilicus \nwithout connection to abdom inal organs. Histopatho-\nlogical examination showed d ilated glandular structures \nsurrounded by cellular endomet rial-type stroma and deep \nperivascular infiltration of lymphocytes (Fig. 2). According \nto these findings, the umbilical lesion was diagnosed as \nprimary cutaneous endometriosis and it was removed by \nlocal surgical excision. Postoperative period was un-\nremarkable and the patient was followed up for 2 years \nwithout recurrence.\nDISCUSSION\nCutaneous endometriosis repres ents 0.5% to 1.0% of all \npatients with ectopic endometriosis. Less than 30% of cu-\ntaneous endometriosis presents  without prior surgical op-\nerative history, which is termed as primary spontaneous \ncutaneous endometriosis 3. Umbilical endometriosis is \ncomposed 0.4% to 4.0% of all endometriosis, high as \ntwo-fifths of extragenital endometrioric lesions. Moreover, \numbilicus is the most common site of primary cutaneous \nendometriosis\n6. Umbilical endometriosis occurs in female \nof reproductive age and associated symptoms are cyclic \npain, bleeding and swelling of the lesion according to the \nmenstrual cycle4.\nSeveral possible pathogenesis of umbilical endometriosis \nwere suggested by multiple investigators. The most com-\nmonly accepted mechanisms are lymphatic or vascular \nmigration, cellular metaplasia, and iatrogenic metastasis\n5. \nSuggested theory includes migr ation of endometrial tissue \nfrom retrogression of menstr uation. Survival of endo-\nmetrial implants after implantation may depend on local \nand systemic factors. Inflamma tory process is then stimu-\nlated by microvascular endothelial injury. Accordingly, it \nmight enhance adhesion of tissu e implants in outside of \nendometrial tissues via production of adhesion molecules \nsuch as integrin and e-cadherins\n7. Major etiologic patho-\ngenesis of secondary umbilical endometriosis could be ex-\nplained by iatrogenic metastasis, endometrial cells implant \nin scars after surgery. In comparison, primary umbilical \nendometriosis may be explained by the theory of vascular \nor lymphatic migration.\n\nPrimary Cutaneous Endometriosis of Umbilicus\nVol. 29, No. 5, 2017 623\nTable 1. Literature review of primary umbilical endometriosis\nReference Age at \ndiagnosis (yr)\nAge at \ninitial (yr) Initial diagnosis Presenting symptom Treatment\nTheunissen and IJpma 9 47 47 Umbilical \nhernia\nAsymptomatic umbilical \nnodule\nSurgical excision\nCalagna et al. 10 33 33 Umbilical \ngranuloma\nSpontaneous catamenial \nbleeding\nSurgical excision\nChikazawa et al. 11 46 44 Swelling during mentrual \nperiod\nSurgical excision\nChikazawa et al. 11 27 23 Pain during \nmenstrual period\nSurgical excision\nPariza and Mavrodin 12 26 25 Pain and discharge Surgical excision\nParamythiotis et al. 13 46 - Uterine \nleiomyoma\nAbdominal and pelvic pain Total hysterectomy with \nexcision of nodule\nGhosh and Das 14 33 33 Umbilical \nendometriosis\nCyclic pain and swelling Excisional biopsy\nGin et al. 15 31 31 Swelling during mentrual \nperiod\nSurgical excision\nKahlenberg \nand Laskey 16\n24 20 Abcess Bloody discharge during \nmenstrual period\nSurgical excision\nFancellu et al. 17 24 24 Umbilical \nendometriosis\nConcomittant bleeding on \nmenstruation\nSurgical excision\nJaime et al. 3 33 33 Spontaneous bleeding Not described\nEfremidou et al. 18 44 38 ∼39 Granuloma Pain during \nmenstrual period\nSurgical excision\nKesici et al. 19 38 38 Omphalitis Umbilical secretion \nand mass\nSurgical excision\nFernández-Aceñero \nand Córdova 4\n38 35 Umbilical \nendometriosis with \nuterine fibroids\nCyclic pain Abdominal hysterectomy \nwith excision of nodule\nDadhwal et al. 8 42 42 Cyclic pain and blackish \ndiscoloration\nSurgical excision\nBagade \nand Guirguis 20\n35 35 Umbilical \nendometriosis\nSpontaneous and \ncyclic bleeing\nGoserelin acetate, and \nthen surgical excision\nVictory et al. 6 47 46 Umbilical bleeding Surgical excision\nBoesgaard-Kjer \net al. 21\n28.5 \n(mean age \nof 10 patients)\n- Periodic color change \nand tenderness\nSurgical excision\nWiegratz et al. 22 27 25 Umbilical \nendometriosis\nIncreasing cyclic pain Oral contraceptive, and \nthen surgical excision\nTaniguchi et al. 23 45 42 Umbilical \nendometriosis\nPainful umbilical mass Surgical excision\nChew et al. 24 44 44 Umbilical \nendometriosis\nProgressively enlarging \numbilical nodule\nGnRH analogue \nleuprorelin acetate\nClaas-Quax et al. 25 27 27 Catamenial bleeding Surgical excision\nSidani et al. 26 37 37 Cyclic swelling \nand discharge\nSurgical excision\nSengupta et al. 27 29 28 Painful nodule Excisional biopsy\nMinaidou et al. 1 26 - Umbilical \nhernia\nUmbilical pain and dark \npurplish nodule\nSurgical excision\nWeng and Yang 5 37 - Cyclic bleeding Gestrinone, and then \nsurgical excision\nKim et al. 28 42 42 Epidermal cyst Size increase and pain Excisional biopsy\nSong et al. 29 25 23 Dermatofibroma Size increase, pain, \ndiscoloration\nSurgical excision\nKyamidis et al. 30 37 27 Umbilical \nendometriosis\nTenderness and \noccasional bleeding\nNot described\n\nSH Loh, et al\n624 Ann Dermatol\nTwenty-nine published studies with primary umbilical en-\ndometriosis were identified in the literature written in \nEnglish and Korean language during the period 2000 ∼\n2016 (Table 1) 1,3-6,8-30. Primary umbilical endometriosis is \ninitially very rare condition, but it is now increasing in \nnumber. Based on all the reports, the mean age of patients \nwas 35.1 years. \nDifferential diagnosis of umbilical endometriosis includes \nkeloid, metastasis of visceral carcinoma, which is referred \nas Sister Mary Joseph nodule and melanoma\n3. Therefore, \nphysicians should work on imaging studies such as ultra-\nsonography or CT or magnetic resonance imaging. Further-\nmore, diagnosis must be con firmed histopathologically to \nexclude malignancy. More importantly, keloid is clinically \nvery similar to umbilical endometriosis. Clinicians should \npay particular attention to patients, especially history of \nsurgery or trauma, and presenting symp toms that are re-\nlated to menstrual cycle. If treatment with steroid intrale-\nsional injection does not improve the symptom, umbilical \nendometriosis should be consid ered for differential diag-\nnosis.\nSurgical excision is the de finitive treatment. Hormonal \ntherapy with gonadotropin-releasing hormone agonists, or-\nal contraceptive and danazol can be used before surgical \nexcision to decrease the size of the lesion and make symp-\ntom relief\n8,31. Recurrence rate is very rare9. In our case, the \nlesion was confirmed by umbi lical ultrasonography and \nabdominal CT and histopathological finding, and removed \nby local surgical excision. \nIn conclusion, cutaneous e ndometriosis of umbilicus \nshould now be recognized as a primary or metastatic pre-\nsentation or iatrogenic complication of endometriosis. \nPatients with primary umbilical endometriosis should un-\ndergo careful history and physi cal examination to rule out \npotential malignancies. Moreover, differential diagnosis \nwith keloid is very important. If the lesion diagnosed with \nkeloid has cyclic symptoms with menstrual period, and \ndoes not improve with treatment, umbilical endometriosis \nshould be suspected. Surgical excision is the treatment of \nchoice to prevent recurrence and to reduce the risk of ma-\nlignant transformation.\nCONFLICTS OF INTEREST\nThe authors have nothing to disclose.\nREFERENCES\n1. Minaidou E, Polymeris A, Vassiliou J, Kondi-Paphiti A, \nKaroutsou E, Katafygiotis P, et al. Primary umbilical \nendometriosis: case report and literature review. Clin Exp \nObstet Gynecol 2012;39:562-564.\n2. Yuen JS, Chow PK, Koong HN, Ho JM, Girija R. Unusual \nsites (thorax and umbilical hernial sac) of endometriosis. J R \nColl Surg Edinb 2001;46:313-315.\n3. Jaime TJ, Jaime TJ, Ormiga P, Leal F, Nogueira OM, \nRodrigues N. Umbilical endometriosis: report of a case and \nits dermoscopic features. An Bras Dermatol 2013;88:121- \n124.\n4. Fernández-Aceñero MJ, Córdova S. 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