Acta Dermato-Venereologica 99-13CompleteContent | Page 28

1299 SHORT COMMUNICATION Epstein-Barr Virus-related Multi-site Mucocutaneous Ulcer: A Previously Undescribed Clinical Subset of a Rare Disease Emilie SCHWOB 1 , Vanessa SZABLEWSKI 2 , Mathilde LERISSON 1 and Olivier DEREURE 1 * 1 Department of Dermatology, Hôpital Saint-Eloi, University of Montpellier and INSERM U1058, 80 avenue Augustin Fliche, Montpellier Cedex 5, and 2 Laboratory of Pathology, University of Montpellier, Montpellier, France. *E-mail: [email protected] Accepted Aug 12, 2019; E-published Aug 13, 2019 Epstein-Barr virus-related mucocutaneous ulcer (EBV+MCU) is a rare and recently individualized type of EBV-induced B-cell lymphoproliferative disorder, most often linked to immunosuppression. It presents mainly as unique or few mucosal and/or cutaneous ulceration(s), initially reported with an indolent, usually self-resolving course unlike other EBV-driven lymphoproliferative conditions. However, more recent reports emphasized its possible clinical heterogeneity, including multisite lesions along with a relatively more aggressive outcome in some cases. We hereby report a further observation of this rare disease that might enlarge its clinical spectrum owing to an unprecedented pattern of atypical and dis- seminated lesions. Fig. 1. Multiple purplish ulcerated nodular lesions on the left thigh. CASE REPORT A 72-year-old male patient with no significant medical background was referred for evaluation of multiple purplish, ulcerated nodular lesions of the left thigh of 2-months duration, rapidly followed by the occurrence on similar elements on the left knee (Fig. 1) and of subcutaneous nodules located on the left shoulder, left ankle and lower back. Apart from these skin lesions, the size of which ranged from 5 to 20 mm in diameter at initial evaluation, physical examination was otherwise unremarkable with a conserved general status and no palpable lymphadenopathy. Standard biological tests showed an isolated moderate lymphopaenia (910/mm 3 ) and mono- clonal IgM kappa. Magnetic resonance imaging (MRI) of the left thigh revealed multiple cutaneous and subcutaneous hypersignals with peripheral enhancement and deep fascia invasion, whereas computed tomography (CT)- 18 F fluorodeoxyglucose positron emis- sion tomography (PET) identified several hyper-metabolic subcu- taneous and muscle foci of the lower limbs, the majority of which did not correspond to clinically relevant elements. Biopsies of left thigh ulceration and left ankle subcutaneous nodule both revealed a heavy dermal infiltrate, consisting of epithelioid histiocytes and polymorphonuclear leukocytes associated with atypical large Hodgkin-like, sometimes mummified cells, displaying irregular and often nucleolated nuclei along with a vastly ulcerated overly- ing epidermis for the thigh lesion (Fig. 2a). Plasmacytoid apoptotic Fig. 2. (a) Thigh skin biopsy (haematoxylin and eosin staining, ×20 magnification; ×40 magnification for the highly magnified inset): heterogeneous admixed dermal infiltrate of large atypical lymphocytes with occasional Reed-Sternberg- like cells, small lymphocytes, and histiocytes. (b) Strong expression of CD30 by Reed-Sternberg-like cells (×10 original magnification). (c) Expression of OCT 2 by Hodgkin-like cells (×20 original magnification). (d) Highly positive in situ hybridization for EBER (×40 original magnification). This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3290 Acta Derm Venereol 2019; 99: 1299–1300