Coexistence of cutaneous endometriosis and ovarian endometrioma: a case report

Background: Umbilical endometriosis is a rare entity accounting for 0.5–4% of cases with endometriosis. Case presentation: Here we report a rare case of umbilical endometriosis with concurrent ovarian endometriomas in a 37-year old primiparous Iranian woman.

This interesting coexistence reflects the importance of thorough gynecological assessment in patients with cutaneous endometriosis to enable appropriate management.

Cutaneous endometriosis, Umbilical endometriosis, Endometrioma, Ovarian cyst © The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. The Creative Commons Public Domain Dedication waiver (http:// creat iveco mmons. org/ publi cdoma in/ zero/1. 0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Endometriosis is a common gynecologic condition characterized by the presence of endometrial tissue in anatomical sites other than the uterus and can lead to chronic pelvic pain or even infertility in women. The most common sites of involvement are ovaries followed by the Douglas pouch and pelvic ligaments, respec - tively [1]. It can be rarely found in other organs such as skin. Umbilical endometriosis is an unusual condition accounting for 0.5–1% of extra pelvic endometriosis [2]. Here we report a case of umbilical endometriosis with concurrent ovarian endometriomas. Case presentation A 37-year old primiparous Iranian woman presented to our dermatology clinic with a complaint of an asympto - matic lesion in the umbilicus. The lesion appeared 1.5 years ago and had slightly increased in size. She denied previous piercing or trauma. However, she had had a laparoscopic cholecystectomy with concurrent ovarian dermoid cystectomy about 10 years ago. She had regular menstrual cycles with normal flow. Physical examination revealed a well-circumscribed, firm, bilobulated 20 × 22 mm2, nontender nodule on her umbilicus (Fig.  1a). An incisional biopsy was performed with differential diagnosis of adnexal tumor, umbilical granuloma, and metastasis, but surprisingly, histopatho - logic assessment showed the presence of a few dilated glands with stratified columnar epithelium in secretory phase (Fig.  2). The glands were surrounded by hyper - cellular stroma. Immunohistochemistry (IHC) staining showed positivity for estrogen receptor in the nuclei of epithelial cells lining glandular structure endometrial-like cells, CD10 diffuse and intense positivity in the stroma, and Ki67 positivity below 1% of epithelial cell nuclei (Fig. 3). These findings confirmed the diagnosis of cuta - neous endometriosis. No signs of atypia or malignancy were observed. After consultation with gynecology ser - vice, abdominopelvic sonography was conducted, which showed an increased density at the umbilicus and also multiple ovarian cysts with appearance compatible with endometrioma (two 15  ×  10 mm2 cysts in right ovary and a 35 × 48  mm2 cyst in left one). After establishing the diagnosis, total umbilec - tomy was suggested. However, due to the patient’s phobia regarding that, just the nodule was excised Open Access *Correspondence: [email protected]; [email protected]; [email protected] 2 Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran Full list of author information is available at the end of the article Page 2 of 3Mohaghegh et al. Journal of Medical Case Reports (2022) 16:256 as completely as possible by dermatologist, and oral progestin (Dienogest) was initiated. The remnants of the umbilical lesion had dramatically improved after 1 month (Fig. 1 b). Follow-up is in progress.

Endometriosis is a benign disease with incidence of 6–10% in women of childbearing age [1 ], usually being seen in the pelvic area. Cutaneous endometriosis can appear in less than 5% of cases [2 ], most of which have a history of former surgery [3 ]. Umbilical endometriosis appeared in 0.5–4% of affected cases, usually presenting with painful umbilical mass with periodic discharge or bleeding. An interesting feature of this case is that the lesion was totally asymptomatic. Another prominent feature is the presence of bilateral ovarian endome - trioma, which reveals the importance of gynecological assessment in patients with cutaneous endometriosis to identify any pelvic involvement, which is reported to occur in 15% of these patients [4 ]. Regarding treatment of cutaneous endometriosis, complete excision is considered as the treatment of choice with or without hormonal therapy for ameliorat - ing the symptoms [5 – 7]. Ovaries are the most common sites of endometriosis [8], and ovarian endometrioma accounts for 35% of all benign ovarian cysts [9 ]. Interestingly, ovarian endo - metriomas are more frequent in the left versus right ovary, possibly due to anatomical asymmetry and com - pression leading to venous congestion and hypoxia in the left side, affecting release of cytokines and sex hor - mones [10, 11]. In our case, endometriomal cyst in left ovary was considerably larger than on the other side, which might be in favor of the above-mentioned theory. Fig. 1 A bilubolated umbilical mass, before (a) and after (b) treatment Fig. 2 Endometrial glandular structures surrounded by scant hypercellular stroma. a H&E ×40, b H&E ×100 Fig. 3 Immunohistochemistry staining for estrogen receptor (×40, ×100) (a, b) and CD10 (×40, ×100) (c, d) Page 3 of 3 Mohaghegh et al. Journal of Medical Case Reports (2022) 16:256 • fast, convenient online submission • thorough peer review by experienced researchers in your field • rapid publication on acceptance • support for research data, including large and complex data types • gold Open Access which fosters wider collaboration and increased citations maximum visibility for your research: over 100M website views per year • At BMC, research is always in progress. Learn more biomedcentral.com/submissions Ready to submit y our researc hReady to submit y our researc h ? Choose BMC and benefit fr om: ? Choose BMC and benefit fr om: In addition to pain, discomfort, and fertility issues, an increased risk of infection, rupture, or transformation into ovarian cancer [12] obligates surgical intervention in larger ovarian endometriomas [9]. Nonsteroidal anti - inflammatory drugs, gonadotropin-releasing hormone (GnRH) agonists, and progestins are also considered as mainstream therapeutic options [9].

Umbilical endometriosis is a rare entity that might occur with concurrent pelvic endometriosis. Hence, thorough gynecological assessment is necessary in such patients to enable proper management.

We acknowledge the staff of Alzahra Dermatology Clinic, with special thanks to Parto Noor Aseman company (www. parnoa. ir) staff, who further observed and scanned stained skin sections using microvisioneer manual whole-slide imaging. Author contributions FM: visiting the patient. PH: writing drafts. PR: histopathological evaluation. ZA: supervision. All listed authors participated equally in preparing the manu- script. All authors read and approved the final manuscript. Funding None. Availability of data and materials The data that support the findings of this study are available from the cor- responding author upon reasonable request. Declarations Ethics approval and consent to participate Ethical approval from the Medical Ethics Committee of Isfahan University of Medical Sciences was provided. Consent for publication Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests The authors declare that they have no competing interests. Author details 1 Department of Dermatology, Skin Diseases and Leishmaniasis Research Center, Isfahan University of Medical Sciences, Isfahan, Iran. 2 Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. 3 Department of Pathology, Isfahan University of Medical Sciences, Isfahan, Iran. 4 Department of Dermatology, Babol University of Medi- cal Sciences, Babol, Iran. Received: 28 September 2021 Accepted: 8 June 2022

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